Aysel ZENGİN, Sara Şebnem KILIÇ

7375

Periyodik Atefl, Aftöz Stomatit, Farenjit, Servikal Adenit Sendromu (PFAPA Sendromu)

PFAPA sendromu etyolojisi bilinmeyen, ani başlayan yüksek ateş, aftöz stomatit, farenjit ve servikal lenfadenopati atakları ile seyreden ve tekrarlayıcı özellik gösteren birhastalıktır. Genellikle beş yaşından küçüklerde ve erkeklerde daha sık görülen busendrom selim seyirlidir. Uzun dönemde sekel gelişmez. Klinik tablo oldukça iyitanımlanmasına karşın hastalığa özgü laboratuvar bulgularının olmaması tanıyıgüçleştirmektedir. Tedavide steroid ve bazı vakalarda tonsillektomi uygulanmaktadır
Anahtar Kelimeler:

PFAPA sendromu, aftöz stomatit, farenjit, lenfadenit

Periodic Fever Accompanied by Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome (PFAPA Syndrome)

PFAPA syndrome is a chronic disease of unknown etiology characterized by suddenonset of high fever, aphthous stomatitis, pharyngitis and cervical lymphadenopathy.This syndrome, which usually observes under 5 years of age and common in boys, hasa benign course and with no long-term sequelae. Although clinical findings are clearenough, as there is no specific test for these diseases, it is sometimes hard to diagnose.Treatment options; steroid therapy and sometimes tonsillectomy
Keywords:

PFAPA syndrome, aphthous stomatitis, pharyngitis, lymphadenitis,

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  • 1. Marshall GS, Edwads KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987; 110:43-6.
  • 2. Frenkel J, Kuis W. Overt and occult rheumatic diseases: the child with chronic fever. Best Pract Res Clin Rheumatol 2002; 16:443-69.
  • 3. Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr 1999; 135:15-21.
  • 4. John CC, Gilsdorf JR. Recurrent fever syndrome in children. Ped Infect Dis J 2002; 21:1071-7.
  • 5. 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.
  • 6. Scholl PR. Periodic fever syndromes. Curr Opin Pediatr 2000; 12:563-6.
  • 7. Aridogan BC, Y›ld›r›m M. Baysal, V Inaloz HS, Baz K, Kaya S. Serum Levels of IL-4 IL-10, IL-12, IL- 13, and IFN-gamma in Behçet’s diseaes. J Dermatol 2003; 31:236-9.
  • 8. Tasher D, Stein M, Dalal I, Somekh E. Colchicine prophylaxis for frequent periodic fever, aphthous stomatitis, pharyngitis and adenitis episodes. Acta Paediatr 2008; 97:1090-2.
  • 9. Dagan E, Gerschoni-Baruh R, Khatib I, Mori A, Brik R. MEFV, TNF1rA, CARD15 and NLRP3 mutation analysis in PFAPA. Rheumatol Int 2009 Jul 5. [Epub ahead of print]
  • 10. Sampaio ICRM, Rodrigo MJ, Marques JGDPM. Two siblings with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA syndrome). Ped Inf Dis J 2009; 28:254-5.
  • 11. Pinto A, Lindemeyer RG, Sollecito TP. The PFAPA syndrome in oral medicine: differential diagnosis and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006; 102:35-9.
  • 12. Feder HM. Jr. Periodic fever, aphthous stomatitis, phryngitis, and adenitis: a clinical review of a new syndrome. Curr Opin Pediatr 2000; 12:253-6.
  • 13. Scully C, Gorsky M, Lozada-Nur F. The diagnosis and management of recurrent aphthous stomatitis: a consensus approach. J Am Dent Assoc 2003; 33:200-7.
  • 14. 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.
  • 15. Long SS. Syndrome of periodic fever, aphthous stomatitis, phryngitis and adenitis (PFAPA): clinial review of a new syndrome. Curr Opin Pediatr 2000; 135:1-5.
  • 16. Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome in adults. IMAJ 2008; 10:358-60.
  • 17. Hernandez-Bou S, Giner M, Plaza AM, Sierra JI, Martin Mateos MA. PFAPA syndrome: with legal to case. Allergol Immunopathol 2003; 31:236-9.
  • 18. Kurtaran H, Karadag A, Catal F, Aktas D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr 2004; 46:354-6.
  • 19. John CC, Gilsdorf JR. Recourrent fever in children. Pediatr Infect Dis J 2002; 21:1071-80.
  • 20. Feder HM JR. Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr Infect Dis J 1992; 11:318-21.
  • 21. Leong SC, Karkos PD, Apostolidou MT. Is there a role fort he otolaryngologist in PFAPA syndrome? A systematic review. Int J Pediatr Otorhinolarygol 2006; 70:1841-5.
Güncel Pediatri-Cover
  • Başlangıç: 2003
  • Yayıncı: Erkan Mor
Arşiv
Sayıdaki Diğer Makaleler

Periyodik Atefl, Aftöz Stomatit, Farenjit, Servikal Adenit Sendromu (PFAPA Sendromu)

Aysel ZENGİN, Sara Şebnem KILIÇ