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 Turk Arch Ped 2009; 44: 80 3 Key words: Aphthous stomatitis periodic fever PFAPA syndrome
Padeh S, Berkun Y. Auto-inflammatory fever syndromes. Rhe- um Dis Clin North Am 2007; 33: 585-623. (Abstract)
Kasapçopur Ö, Arısoy N. Ailesel Akdeniz ateşi ve diğer otoenf- lamatuar hastalıklar. Türk Pediatri Arşivi 2006; 41: 9-17. (Abstract) / (Full Text) / (PDF)
Marshall GS, Edwards KM, Butler I, Lawton AR. Syndrome of periodic fever, pharyngitis and aphthous stomatitis. J Pediatr 1987; 110: 43-6. (Abstract)
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. (Abstract)
Lierl M. Periodic fever syndromes: a diagnostic challenge for the allergist. Allergy 2007; 62: 1349-58. (Abstract)
Feder H. Periodic fever, aphthous stomatitis, pharyngitis, adeni- tis: a clinical review of a new syndrome. Curr Opin Pediatr 2000; 12: 253-6. (Abstract)
Scully C, Hodgson T, Lachmann H. Auto-inflammatory syndro- mes and oral health. Oral diseases 2008; 14: 690-9. (Abstract)
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. (Abstract)
Padeh S, Brezniak N, Zemer D, et al. Periodic fever, aphthous stomatitis, pharyngitis and adenopathy syndrome: clinical cha- racteristics and outcome. J Pediatr 1999; 135: 98-101. (Abstract)
Renko M, Salo E, Putto-Laurila A, et al. A randomized, control- led trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. J Pediatr 2007; 151: 289-92. (Abstract)
Licameli G, Jeffrey J, Luz J, Jones D, Kenna M. Effect of adeno- tonsillectomy in PFAPA syndrome. Arch Otolarnygol Head Neck Surg 2008; 134: 136-40. (Abstract)
Tasher D, Somekh E, Dala I. PFAPA syndrome: new clinical as- pects disclosed. Arch Dis Child 2005; 91: 981-4. (Abstract)
Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, Bitso- ri M, Heldionis ES. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002; 86: 434-5. (Abstract)
Garavello W, Romagnoli M, Gaini RM. Effectiveness of adeno- tonsillectomy in PFAPA syndrome: a randomized study. J Pedi- atr 2009; 155: 250-3. (Abstract)
Kurtaran H, Karadağ A, Çatal F, Aktaş D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr 2004; 48: 354-6. (Abstract) / (Full Text) / (PDF)
Dagan E, Gerschoni-Baruh R, Khatib I, Mori A, Brik R. MEFV, TNF1rA, CARD15 and NLRP3 mutation analysis in PFAPA. Rhe- umatol Int 2009 (e pub 5 july). (Abstract)
Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome in adults. IMAJ 2008; 10: 358-60. (Abstract)
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 Türk Ped Arş 2009; 44: 80 3 Anahtar kelimeler: Aftöz stomatit PFAPA sendromu yineleyen ateş
Padeh S, Berkun Y. Auto-inflammatory fever syndromes. Rhe- um Dis Clin North Am 2007; 33: 585-623. (Abstract)
Kasapçopur Ö, Arısoy N. Ailesel Akdeniz ateşi ve diğer otoenf- lamatuar hastalıklar. Türk Pediatri Arşivi 2006; 41: 9-17. (Abstract) / (Full Text) / (PDF)
Marshall GS, Edwards KM, Butler I, Lawton AR. Syndrome of periodic fever, pharyngitis and aphthous stomatitis. J Pediatr 1987; 110: 43-6. (Abstract)
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. (Abstract)
Lierl M. Periodic fever syndromes: a diagnostic challenge for the allergist. Allergy 2007; 62: 1349-58. (Abstract)
Feder H. Periodic fever, aphthous stomatitis, pharyngitis, adeni- tis: a clinical review of a new syndrome. Curr Opin Pediatr 2000; 12: 253-6. (Abstract)
Scully C, Hodgson T, Lachmann H. Auto-inflammatory syndro- mes and oral health. Oral diseases 2008; 14: 690-9. (Abstract)
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. (Abstract)
Padeh S, Brezniak N, Zemer D, et al. Periodic fever, aphthous stomatitis, pharyngitis and adenopathy syndrome: clinical cha- racteristics and outcome. J Pediatr 1999; 135: 98-101. (Abstract)
Renko M, Salo E, Putto-Laurila A, et al. A randomized, control- led trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. J Pediatr 2007; 151: 289-92. (Abstract)
Licameli G, Jeffrey J, Luz J, Jones D, Kenna M. Effect of adeno- tonsillectomy in PFAPA syndrome. Arch Otolarnygol Head Neck Surg 2008; 134: 136-40. (Abstract)
Tasher D, Somekh E, Dala I. PFAPA syndrome: new clinical as- pects disclosed. Arch Dis Child 2005; 91: 981-4. (Abstract)
Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, Bitso- ri M, Heldionis ES. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002; 86: 434-5. (Abstract)
Garavello W, Romagnoli M, Gaini RM. Effectiveness of adeno- tonsillectomy in PFAPA syndrome: a randomized study. J Pedi- atr 2009; 155: 250-3. (Abstract)
Kurtaran H, Karadağ A, Çatal F, Aktaş D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr 2004; 48: 354-6. (Abstract) / (Full Text) / (PDF)
Dagan E, Gerschoni-Baruh R, Khatib I, Mori A, Brik R. MEFV, TNF1rA, CARD15 and NLRP3 mutation analysis in PFAPA. Rhe- umatol Int 2009 (e pub 5 july). (Abstract)
Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome in adults. IMAJ 2008; 10: 358-60. (Abstract)