Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; A population-based study

Acta Paediatrica, International Journal of Paediatrics

Volumn 102, Issue 2, 2013, Pages 187-192

  Førsvoll, Jostein ^a   Kristoffersen, Einar Klæboe ^b,c   Øymar, Knut ^a,b  

^a STAVANGER UNIVERSITY HOSPITAL   (Norway)

^b UNIVERSITY OF BERGEN   (Norway)

^c HAUKELAND UNIVERSITY HOSPITAL   (Norway)

Author keywords

Adenitis; Fever; Mouth disease; Pharyngitis; Tonsillectomy

Indexed keywords

ANTIBIOTIC AGENT; IBUPROFEN; PARACETAMOL;

ADENOIDECTOMY; ANTIBIOTIC THERAPY; ARTICLE; CHILD; CLINICAL ARTICLE; FEMALE; FEVER; FOLLOW UP; HUMAN; INCIDENCE; INFANT; INTERVIEW; MALE; NORWAY; ONSET AGE; OUTCOME ASSESSMENT; PATIENT SATISFACTION; PFAPA SYNDROME; PRESCHOOL CHILD; PRIORITY JOURNAL; PROSPECTIVE STUDY; RECURRENT FEVER; RELAPSE; SCHOOL CHILD; THROAT INFECTIO; TONSILLECTOMY;

AGE OF ONSET; CHILD, PRESCHOOL; FEMALE; FEVER; FOLLOW-UP STUDIES; HUMANS; INCIDENCE; INFANT; LYMPHADENITIS; MALE; NECK; NORWAY; PHARYNGITIS; PROSPECTIVE STUDIES; STOMATITIS, APHTHOUS; SYNDROME; TONSILLECTOMY; TREATMENT OUTCOME;

EID: 84872288485     PISSN: 08035253     EISSN: 16512227     Source Type: Journal    
DOI: 10.1111/apa.12069     Document Type: Article

References (30)

1. Thomas KT, Feder HM Jr, Lawton AR, Edwards KM,. Periodic fever syndrome in children. J Pediatr 1999; 135: 15-21.
2. Lierl M,. Periodic fever syndromes: a diagnostic challenge for the allergist. Allergy 2007; 62: 1349-58.
3. Marshall GS, Edwards KM, Butler J, Lawton AR,. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987; 110: 43-6.
4. Masters SL, Simon A, Aksentijevich I, Kastner DL,. Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease. Annu Rev Immunol 2009; 27: 621-68.
5. Stojanov S, Lapidus S, Chitkara P, Feder H, Salazar JC, Fleisher TA, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Proc Natl Acad Sci USA 2011; 108: 7148-53.
6. Stojanov S, Hoffmann F, Kery A, Renner ED, Hartl D, Lohse P, et al. Cytokine profile in PFAPA syndrome suggests continuous inflammation and reduced anti-inflammatory response. Eur Cytokine Netw 2006; 17: 90-7.
7. Brown KL, Wekell P, Osla V, Sundqvist M, Savman K, Fasth A, et al. Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. BMC Pediatr 2010; 10: 65.
8. Abramson JS, Givner LB, Thompson JN,. Possible role of tonsillectomy and adenoidectomy in children with recurrent fever and tonsillopharyngitis. Pediatr Infect Dis J 1989; 8: 119-20.
9. Padeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. J Pediatr 1999; 135: 98-101.
10. Dahn KA, Glode MP, Chan KH,. Periodic fever and pharyngitis in young children: a new disease for the otolaryngologist? Arch Otolaryngol Head Neck Surg 2000; 126: 1146-9.
11. Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, Bitsori M, Helidonis ES,. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002; 86: 434-5.
12. Berlucchi M, Meini A, Plebani A, Bonvini MG, Lombardi D, Nicolai P, et al. Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature. Ann Otol Rhinol Laryngol 2003; 112: 365-9.
13. Tasher D, Somekh E, Dalal I,. PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child 2006; 91: 981-4.
14. Renko M, Salo E, Putto-Laurila A, Saxen H, Mattila PS, Luotonen J, et al. A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr 2007; 151: 289-92.
15. Licameli G, Jeffrey J, Luz J, Jones D, Kenna M, Licameli G, et al. Effect of adenotonsillectomy in PFAPA syndrome. Arch Otolaryngol Head Neck Surg 2008; 134: 136-40.
16. Wong KK, Finlay JC, Moxham JP, Wong KK, Finlay JC, Moxham JP,. Role of Tonsillectomy in PFAPA Syndrome. Arch Otolaryngol Head Neck Surg 2008; 134: 16-9.
17. Garavello W, Romagnoli M, Gaini RM, Garavello W, Romagnoli M, Gaini RM,. Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J Pediatr 2009; 155: 250-3.
18. Peridis S, Koudoumnakis E, Theodoridis A, Stefanaki K, Helmis G, Houlakis M,. Surgical outcomes and histology findings after tonsillectomy in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. Am J Otolaryngol 2010; 31: 472-5.
19. Pignataro L, Torretta S, Pietrogrande MC, Dellepiane RM, Pavesi P, Bossi A, et al. Outcome of tonsillectomy in selected patients with PFAPA syndrome.[Erratum appears in Arch Otolaryngol Head Neck Surg. 2009 Sep;135(9):952]. Arch Otolaryngol Head Neck Surg 2009; 135: 548-53.
20. Feder HM, Salazar JC,. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr 2010; 99: 178-84.
21. Statistics Norway 2012. Available from: http://www.ssb.no/befolkning-en/.
22. John CC, Gilsdorf JR,. Recurrent fever in children. Pediatr Infect Dis J 2002; 21: 1071-7.
23. Long SS,. Distinguishing among prolonged, recurrent, and periodic fever syndromes: approach of a pediatric infectious diseases subspecialist. Pediatr Clin North Am 2005; 52: 811-35, vii.
24. Burton MJ, Pollard AJ, Ramsden JD,. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev 2010; 9: CD008669.
25. Garavello W, Pignataro L, Gaini L, Torretta S, Somigliana E, Gaini R,. Tonsillectomy in children with periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr 2011; 159: 138-42.
26. Padeh S, Stoffman N, Berkun Y, Padeh S, Stoffman N, Berkun Y,. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults. Isr Med Assoc J 2008; 10: 358-60.
27. Vestergaard H, Wohlfahrt J, Westergaard T, Pipper C, Rasmussen N, Melbye M,. Incidence of tonsillectomy in Denmark, 1980 to 2001. Pediatr Infect Dis J 2007; 26: 1117-21.
28. Pelagatti MA, Meini A, Caorsi R, Cattalini M, Federici S, Zulian F, et al. Long-term clinical profile of children with the low-penetrance R92Q mutation of the TNFRSF1A gene. Arthritis Rheum 2011; 63: 1141-50.
29. Gattorno M, Caorsi R, Meini A, Cattalini M, Federici S, Zulian F, et al. Differentiating PFAPA syndrome from monogenic periodic fevers. Pediatrics 2009; 124: e721-8.
30. Forsvoll JA, Oymar K,. C-reactive protein in the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Acta Paediatr 2007; 96: 1670-3.