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PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, (PFAPA) SYNDROME AND NLRP3 GENE ASSOCIATION

Cilt: 45 Sayı: 2 30 Haziran 2023
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PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, (PFAPA) SYNDROME AND NLRP3 GENE ASSOCIATION

Abstract

PFAPA syndrome, which also includes aphthous stomatitis, pharyngitis, and adenitis, is the most common type of recurrent fever in children. Usually happens before the age of five. This syndrome is characterized by attacks lasting 3-7 days, recurring every 2-8 weeks with high fever (39 C and above) accompanied by at least one of the signs of aphthous stomatitis, pharyngitis, and/or cervical adenitis. Between attacks, the child is completely healthy.PFAPA is a benign disease that regresses with age. Steroids are used in the treatment of attacks. Colchicine is often preferred in prophylactic treatment. However, if there is no response to medical treatment, surgery (tonsillectomy) can be performed. Genetic and environmental factors are considered in the etiology. Genetic susceptibility concentrated on the genes for Familiar Mediterranean Fever (FMF, MEFV), TNF-Receptor-Associated Periodic Syndrome (TRAPS, gene TNFRF1A), HyperIgDSyndrome (HIDS, gene MVK), and Cryopyrin-Associated Periodic Syndrome (CAPS, gene NLRP3). But its etiology is still unknown.

Keywords

Destekleyen Kurum

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Proje Numarası

yok

Kaynakça

  1. 1. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110(1):43–6.
  2. 2. Marshall GS, Edwards KM, Lawton AR. PFAPA syndrome. Pediatr Infect Dis J. 1989;8(9):658–9.
  3. 3. Ter Haar N, Lachmann H, Ozen S, Woo P, Uziel Y, Modesto C, et al. Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review. Ann Rheum Dis. 2013;72(5):678–85.
  4. 4. Forsvoll J, Kristoffersen EK, Oymar K. Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome; a population-based study. Acta Paediatr. 2013;102(2):187–92.
  5. 5. Hofer M, Pillet P, Cochard MM, et al. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Rheumatology (Oxford). 2014;53:1125e1129.
  6. 6. Vanoni F, Theodoropoulou K, Hofer M. PFAPA syndrome: a review on treatment and outcome. Pediatr Rheumatol Online J. 2016;14:38.
  7. 7. Ferreira Cristina S, Costa A, Toscano M, Kakoo Brioso E, Cipriano P. Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: a rare etiology of fever in adults. Cureus. 2021;13(4):e14749 .
  8. 8. Theodoropoulou K, Vanoni F, Hofer M. Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome: a Review of the Pathogenesis. Curr Rheumatol Rep. 2016 Apr;18(4):18.

Ayrıntılar

Birincil Dil

İngilizce

Konular

Aile Hekimliği

Bölüm

Derleme

Erken Görünüm Tarihi

30 Haziran 2023

Yayımlanma Tarihi

30 Haziran 2023

Gönderilme Tarihi

6 Haziran 2023

Kabul Tarihi

25 Haziran 2023

Yayımlandığı Sayı

Yıl 2023 Cilt: 45 Sayı: 2

Kaynak Göster

AMA
1.Ünsal G. PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, (PFAPA) SYNDROME AND NLRP3 GENE ASSOCIATION. CMJ. 2023;45(2):1-6. doi:10.7197/cmj.1310396