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PFAPA Sendromu (Periyodik Ateş, Aftöz Stomatit, Farenjit ve Adenit Birlikteliği)

Year 2017, Volume: 11 Issue: 1, 59 - 63, 20.03.2017

Ayşe Nur Topuz Selin Kızılgök Mahir Serbes Nafiz Bozdemir

https://doi.org/10.21763/tjfmpc.295947

Abstract

PFAPA sendromu; tekrarlayıcı, aftöz stomatit, farenjit ve servikal lenfadenopati ataklarının eşlik ettiği etyolojisi henüz tam olarak bilinmeyen ateşli bir hastalıktır. Benign karakterde olup beş yaşından küçüklerde ve erkek cinsiyette daha sık görülür. Uzun dönemde sekel gelişmez. Hastalığın klinik tablosu spesifik olmasına rağmen laboratuvar bulgularının non-spesifik olması nedeniyle tanısı güçtür. Bu olgu sunumunda kliniğe başvuran ve PFAPA tanısı konulan iki hasta tartışıldı.

Keywords

PFAPA sendromu aftöz stomatit farenjit lenfadenit

References

  • 1. Murata T, Okamoto N, Shimizu T, Tamai H. Diagnosis and management of periodic fever with aphthous pharyngitis and adenitis (PFAPA). Nihon Rinsho Meneki Gakkai Kaishi 2007;30(2):101-7.
  • 2. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987;110(1):43-6.
  • 3. Kasapçopur Ö, Arısoy N. PFAPAsendromu. Turk Arch Ped 2009;44(3):80-3.
  • 4. John CC, Gilsdorf JR. Recurrent fever syndrome in children. Ped Infect Dis J 2002; 21:1071-7.
  • 5. Scholl PR. Periodic fever syndromes. Curr Opin Pediatr 2000; 12:563-6.
  • 6. 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.
  • 7. 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.
  • 8. Feder H. Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome. Curr Opin Pediatr 2000;12(3):253-6.
  • 9. 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(1):35-9.
  • 10. Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr 1999;135(1):15-21.
  • 11. Król P, Böhm M, Sula V, Dytrych P, Katra R, Nemcová D, et al. PFAPA syndrome: clinical characteristics and treatmentoutcomes in a large single-centre cohort. Clin Exp Rheumatol 2013;31(6):980–7.
  • 12. Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied byaphthous stomatitis, pharyngitis and cervical adenitissyndrome (PFAPA syndrome) in adults. Isr Med Assoc J 2008;10(5):358–60. PMID: 18605359.
  • 13. Terreri MT, Bernardo WM, Len CA, da Silva CA, de Magalhães CM, Sacchetti SB, et al. Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Rev Bras Reumatol Engl Ed 2016 Jan-Feb;56(1):52-7.
  • 14. Ter Haar NM, Oswald M, Jeyaratnam J, Anton J, Barron KS, Brogan PA, et al. Recommendations for the management of autoinflammatory diseases. Ann Rheum Dis 2015; 74:1636–44.
  • 15. Hernandez-Bou S, Giner M, Plaza AM, Sierra JI, Martin Mateos MA. PFAPA syndrome: with legal to case. Allergol Immunopathol 2003; 31:236-9.
  • 16. Kurtaran H, Karadag A, Catal F, Aktas D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr 2004; 6:354-6.

PFAPA Syndrome (Periodic fever, Aphthous stomatitis, Pharyngitis and Adenitis)

Year 2017, Volume: 11 Issue: 1, 59 - 63, 20.03.2017

Ayşe Nur Topuz Selin Kızılgök Mahir Serbes Nafiz Bozdemir

https://doi.org/10.21763/tjfmpc.295947

Abstract

PFAPA syndrome is a febril disease with unknown etiology which is
characterized by reccurrent aphthous stomatitis, pharyngitis and servical
adenitis. It has a benign clinical course and frequently seen in male patients
under five-year-old. Although the clinical findings of PFAPA is specific, its
diagnosis is often diffucult due to non-specific laboratory findings. In this report,
we discuss two patients diagnosed with PFAPA syndrome.


PFAPA sendromu; tekrarlayıcı, aftöz
stomatit, farenjit ve servikal lenfadenopati ataklarının eşlik ettiği
etyolojisi henüz tam olarak bilinmeyen ateşli bir hastalıktır.  Benign karakterde olup beş yaşından
küçüklerde ve erkek cinsiyette daha sık görülür. Uzun dönemde sekel gelişmez.
Hastalığın klinik tablosu spesifik olmasına rağmen laboratuvar bulgularının
non-spesifik olması nedeniyle tanısı güçtür. Bu olgu sunumunda kliniğe başvuran
ve PFAPA tanısı konulan iki hasta tartışıldı.  

Keywords

PFAPA syndrome aphthous stomatitis pharyngitis adenitis

References

  • 1. Murata T, Okamoto N, Shimizu T, Tamai H. Diagnosis and management of periodic fever with aphthous pharyngitis and adenitis (PFAPA). Nihon Rinsho Meneki Gakkai Kaishi 2007;30(2):101-7.
  • 2. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987;110(1):43-6.
  • 3. Kasapçopur Ö, Arısoy N. PFAPAsendromu. Turk Arch Ped 2009;44(3):80-3.
  • 4. John CC, Gilsdorf JR. Recurrent fever syndrome in children. Ped Infect Dis J 2002; 21:1071-7.
  • 5. Scholl PR. Periodic fever syndromes. Curr Opin Pediatr 2000; 12:563-6.
  • 6. 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.
  • 7. 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.
  • 8. Feder H. Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome. Curr Opin Pediatr 2000;12(3):253-6.
  • 9. 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(1):35-9.
  • 10. Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr 1999;135(1):15-21.
  • 11. Król P, Böhm M, Sula V, Dytrych P, Katra R, Nemcová D, et al. PFAPA syndrome: clinical characteristics and treatmentoutcomes in a large single-centre cohort. Clin Exp Rheumatol 2013;31(6):980–7.
  • 12. Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied byaphthous stomatitis, pharyngitis and cervical adenitissyndrome (PFAPA syndrome) in adults. Isr Med Assoc J 2008;10(5):358–60. PMID: 18605359.
  • 13. Terreri MT, Bernardo WM, Len CA, da Silva CA, de Magalhães CM, Sacchetti SB, et al. Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Rev Bras Reumatol Engl Ed 2016 Jan-Feb;56(1):52-7.
  • 14. Ter Haar NM, Oswald M, Jeyaratnam J, Anton J, Barron KS, Brogan PA, et al. Recommendations for the management of autoinflammatory diseases. Ann Rheum Dis 2015; 74:1636–44.
  • 15. Hernandez-Bou S, Giner M, Plaza AM, Sierra JI, Martin Mateos MA. PFAPA syndrome: with legal to case. Allergol Immunopathol 2003; 31:236-9.
  • 16. Kurtaran H, Karadag A, Catal F, Aktas D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr 2004; 6:354-6.
There are 16 citations in total.

Details

Primary Language Turkish
Subjects ​Internal Diseases
Journal Section Case Report
Authors

Ayşe Nur Topuz

Selin Kızılgök This is me

Mahir Serbes This is me

Nafiz Bozdemir

Publication Date March 20, 2017
Submission Date March 2, 2017
Published in Issue Year 2017 Volume: 11 Issue: 1

Cite

Vancouver Topuz AN, Kızılgök S, Serbes M, Bozdemir N. PFAPA Sendromu (Periyodik Ateş, Aftöz Stomatit, Farenjit ve Adenit Birlikteliği). TJFMPC. 2017;11(1):59-63.
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English or Turkish manuscripts from authors with new knowledge to contribute to understanding and improving health and primary care are welcome.