Abstract
PFAPA (periyodik ateş, aftöz stomatit, farenjit ve adenit) sendromu çocuklarda 4-6 gün süren tekrarlayan ateş atakları ile karakterize göreceli olarak daha az bilinen periyodik ateş sendromlarından biridir. Ateşe aftöz stomatit, farenjit ve adenit eşlik eder. FMF (familial Mediterranean fever) ve PFAPA sendromu arasında bir çok klinik benzerlikler bulunmaktadır. Doğru tanı için bu sendromun akla gelmesi gereklidir.Burada FMF ön tanısıyla kolşisin profilaksisi kullanılan 3 yaşında bir kız takdim edilmektedir. İzleminde hasta PFAPA sendromu olarak tanı almıştır. Kolşisin tedavisiyle ateş atakları arasındaki süre uzamıştır. PFAPA sendromuyla FMF arasındaki benzerliklerden dolayı kolşisin tedavisi PFAPA sendromlu çocuklarda etkili bir yaklaşım olarak önerilebilir.
Keywords
References
- Tasher D, Stein M, Dalal I, Somekh E. Colchicine prophylaxis for frequent periodic fever, aphtous stomatitis, pharygitis and adenitis episodes. Acta Paediatr 2008;97(8):1090-2.
- Lierl M. Periodic fever syndromes: a diagnostic challenge for the allergist. Allergy 2007;62(12):1349-58.
- Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphtous stomatitis, pharyngitis and cervical adenitis syndrome in adults. Isr Med Assoc J 2008;10(5):358-60.
- Ataş B, Caksen H, Arslan S, Tuncer O, Kırımi E, Odabaş D. PFA- PA syndrome mimicking familial mediterranean fever: Report of a Turkish child. J Emerg Med 2003;25(4):383-5.
- Wong KK, Finlay JC, Moxham JP. Role of Tonsillectomy in PFA- PA syndrome. Arch Otolaryngol Head Neck Surg 2008;134(1):16- 9.
- Leong SC, Karkos PD, Apostolidou MT. Is there a role for the otorhinolaryngologist in PFAPA syndrome? A systematic review. Int J Pediatr otorhinolaryngol 2006;70(11):1841-5.
- Cazeneuve C, Genevieve D, Amselem S, Hentgen V, Hau I, Reinert P. MEFV gene analysis in PFAPA. J Pediatr 2003;143(1):140-1.
- Dagan E, Baruch RG, Khatib I, Brik MR. MEFV, TNF1rA, CARD15 and NLRP3 mutation analysis in PFAPA. Rheumatol Int 2010;30(5):633-6.
- Kasapçopur Ö, Arısoy N. PFAPA syndrome. Turk Ped Arch 2009;44(3):80-3.
Abstract
PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome is a relatively not wellrecognized periodic fever syndrome characterized by recurrent febrile episodes persisting 4-6 days in children. Fever is accompanied by aphtous stomatitis, pharyngitis, and cervical adenitis. There are several clinical similarities between familial Mediterranean fever (FMF) and PFAPA syndrome. For accurate diagnosis, increased awareness of this syndrome is essential. We describe a three-year-old girl with PFAPA who used colchicine prophylaxis with the presumptive diagnosis of FMF. At the follow-up, she was diagnosed as having PFAPA syndrome. The interval between the febrile episodes was increased from four weeks to 12 weeks after colchicine therapy. Since there are many similarities between the FMF and PFAPA syndrome, colchicine use can be offered as an effective approach for children with PFAPA
Keywords
References
- Tasher D, Stein M, Dalal I, Somekh E. Colchicine prophylaxis for frequent periodic fever, aphtous stomatitis, pharygitis and adenitis episodes. Acta Paediatr 2008;97(8):1090-2.
- Lierl M. Periodic fever syndromes: a diagnostic challenge for the allergist. Allergy 2007;62(12):1349-58.
- Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphtous stomatitis, pharyngitis and cervical adenitis syndrome in adults. Isr Med Assoc J 2008;10(5):358-60.
- Ataş B, Caksen H, Arslan S, Tuncer O, Kırımi E, Odabaş D. PFA- PA syndrome mimicking familial mediterranean fever: Report of a Turkish child. J Emerg Med 2003;25(4):383-5.
- Wong KK, Finlay JC, Moxham JP. Role of Tonsillectomy in PFA- PA syndrome. Arch Otolaryngol Head Neck Surg 2008;134(1):16- 9.
- Leong SC, Karkos PD, Apostolidou MT. Is there a role for the otorhinolaryngologist in PFAPA syndrome? A systematic review. Int J Pediatr otorhinolaryngol 2006;70(11):1841-5.
- Cazeneuve C, Genevieve D, Amselem S, Hentgen V, Hau I, Reinert P. MEFV gene analysis in PFAPA. J Pediatr 2003;143(1):140-1.
- Dagan E, Baruch RG, Khatib I, Brik MR. MEFV, TNF1rA, CARD15 and NLRP3 mutation analysis in PFAPA. Rheumatol Int 2010;30(5):633-6.
- Kasapçopur Ö, Arısoy N. PFAPA syndrome. Turk Ped Arch 2009;44(3):80-3.
Details
| Other ID | JA64UV27DY |
|---|---|
| Journal Section | Research Article |
| Authors | |
| Publication Date | December 1, 2012 |
| Submission Date | December 1, 2012 |
| Published in Issue | Year 2012 Volume: 6 Issue: 1 |
The publication language of Turkish Journal of Pediatric Disease is English.
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