PFAPA sendromu

PFAPA sendromu çocukluk çağında yineleyen ateş, aftöz stomatit, farenjit ve servikal adenitle ortaya çıkan bir otoenflamatuar hastalıktır. Hastalık çoğunlukla beş yaşın altında ortaya çıkar. Hastalığa ait ateş atakları düzensiz aralıklarla yineler ve ortalama 3-4 sürüp, kendiliğinden düzelir. Ateş çocuklarda genellikle 39 derecenin üzerindedir ve tüm antibiyotik ve ateş düşürücü tedavilere yanıtsızdır. Hastalardaki klinik bulguların tümü tek doz kortikosteroid uygulaması ile hemen kaybolur. Kalıcı tedavi seçeneği ise çocuklarda uygulanacak olan tonsilloadenoidektomidir. Bu derlemede çocukluk çağındaki PFAPA hastalığın özellikleri dile getirilmiştir

PFAPA syndrome

PFAPA syndrome is an autoinflammatory disease of the childhood period presenting with recurrent fever, aphthous stomatitis, pharyngitis and cervical lymphadenitis. The disease is often seen in children under the age of five. Fever attacks recur with irregular time intervals and are unresponsive to treatment with antibiotics and antipyretics. All of the clinical findings resolve immediately with a single dose of corticosteroid. The ultimate treatment method is tonsilloadenoidectomy. Main aspects of PFAPA syndrome are mentioned in this review.

PDF

___

1. Padeh S, Berkun Y. Auto-inflammatory fever syndromes. Rheum Dis Clin North Am 2007; 33: 585-623.
2. Kasapçopur Ö, Arısoy N. Ailesel Akdeniz ateşi ve diğer otoenflamatuar hastalıklar. Türk Pediatri Arşivi 2006; 41: 9-17.
3. Marshall GS, Edwards KM, Butler I, Lawton AR. Syndrome of periodic fever, pharyngitis and aphthous stomatitis. J Pediatr 1987; 110: 43-6.
4. Leong SC, Karkos PD, Apostolidou MT. Is there a role for the otolaryngologist in PFAPA syndrome? A systematic review. Int J Pediatr Otorhinolaryngol 2006; 70: 1841-5.
5. Lierl M. Periodic fever syndromes: a diagnostic challenge for the allergist. Allergy 2007; 62: 1349-58.
6. Feder H. Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome. Curr Opin Pediatr 2000; 12: 253-6.
7. Scully C, Hodgson T, Lachmann H. Auto-inflammatory syndromes and oral health. Oral diseases 2008; 14: 690-9.
8. 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.
9. Padeh S, Brezniak N, Zemer D, et al. Periodic fever, aphthous stomatitis, pharyngitis and adenopathy syndrome: clinical characteristics and outcome. J Pediatr 1999; 135: 98-101.
10. Renko M, Salo E, Putto-Laurila A, et al. A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. J Pediatr 2007; 151: 289-92.
11. Licameli G, Jeffrey J, Luz J, Jones D, Kenna M. Effect of adenotonsillectomy in PFAPA syndrome. Arch Otolarnygol Head Neck Surg 2008; 134: 136-40.
12. Tasher D, Somekh E, Dala I. PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child 2005; 91: 981-4.
13. Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, Bitsori M, Heldionis ES. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002; 86: 434-5.
14. Garavello W, Romagnoli M, Gaini RM. Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J Pediatr 2009; 155: 250-3.
15. Kurtaran H, Karadağ A, Çatal F, Aktaş D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr 2004; 48: 354-6.
16. Dagan E, Gerschoni-Baruh R, Khatib I, Mori A, Brik R. MEFV, TNF1rA, CARD15 and NLRP3 mutation analysis in PFAPA. Rheumatol Int 2009 (e pub 5 july).
17. 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.
18. Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome in adults. IMAJ 2008; 10: 358-60.