Journals / Bakırköy Tıp Dergisi / 2008 / Cilt: 4 - Sayı: 3

The clinical and laboratory characteristics in patients with periodic fever, apthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome

Periyodik ateş, aftöz stomatit, farengit ve adenit (PFAPA) sendromlu olgularımızın klinik ve laboratuvar özellikleri

Pages
107–110
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

By this way, early diagnosis would prevent the unnecessary usage of antibiotics.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Objective: The aim of this study is to evaluate the clinical and laboratory findings and results of the treatment in patients with PFAPA.
📊
Key Findings & Data:Results: Eleven patients (8 boys, 3 girls), aged between 26-60 months were evaluated.
💡
Conclusion & Impact:By this way, early diagnosis would prevent the unnecessary usage of antibiotics.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Objective: The aim of this study is to evaluate the clinical and laboratory findings and results of the treatment in patients with PFAPA. Materials and methods: The frequency of the attacks, the age at the onset of the disease, the presence of similar symptoms in the family members, the associated symptoms found during attacks, the laboratory parameters and the results of the treatment in the patients with PFAPA were evaluated retrospectively. Results: Eleven patients (8 boys, 3 girls), aged between 26-60 months were evaluated. Seven of them had at least one parent with recurrent tonsillitis attacks. The complaints of the patients have begun at their ages between 8-36 months; periodic fever lasted in 3-6 days of every 3-7 weeks intervals. The pharyngitis (100%), cervical lymphadenopathy (90%), apthous stomatitis (81.8%) and exudative tonsillitis (45.45%) were found as the common symptoms. In the laboratory studies, WBC’s were 13035+1459/mm3 (11000-15890), CRP was positive, ESR’s were 32+10 mm/h (21-62 mm/h). All throat cultures were found negative for bacteria. In two patients, FMF gene mutations were found. The fever was decreased dramatically in 2-8 hours with the 1-2 mg/kg/dose oral prednisolon therapy, and it did not rise again until the next attack. Results: PFAPA could easily be diagnosed on patients with complaints of recurrent fever and tonsilopharyngitis by taking a good history, careful physical examination and conscious observation. By this way, early diagnosis would prevent the unnecessary usage of antibiotics.

Özet

Amaç: PFAPA sendromu tanısı alan olgularımızın klinik, laboratuar bulguları ve tedavi sonuçlarını değerlendirmek Gereç ve Yöntem: PFAPA tanısı alan hastaların atak sıklığı, başlama yaşı, ailede benzer olguların varlığı, atak sırasında eşlik eden fizik muayane bulguları ve laboratuvar parametreleri ve verilen tedaviye yanıtları retrospektif olarak değerlendirildi. Bulgular: Yaşları 24-60 ay arasında olan 8’i erkek, 3’ü kız 11 hasta değerlendirildi. Yedi olguda ebeveynlerden en az birinde tekrarlayan tonsillit atakları vardı. Hastaların yakınmalarının başlama yaşı 8-36 ay olup, 3-7 hafta ara ile 3-6 gün süren periyodik ateşle birlikte en sık gözlenen bulgular farenjit (%100), servikal lenfadenopati (%90), aftöz stomatit (%81.8) ve eksüdatif tonsillit (%45.45) olarak saptandı. Laboratuvar incelemelerinde beyaz küre 13035±1459/mm3 (11000-15890/mm3), CRP pozitif, eritrosit sedimantasyon hızı 32±10mm/saat, (21-62mm/saat), boğaz kültürlerinde üreme olmadı. İki olguda heterozigot Ailevi Akdeniz Ateşi gen mutasyonu saptandı. Hastaların hepsinde 1-2 mg/kg oral prednizolon ile 2-8 saat içerisinde ateş terleyerek düştü, sonraki atağa kadar tekrar yükselmedi Sonuç: Düzenli aralıklarla tekrarlayan ateş ve tonsillofarenjit yakınması olan hastalarda iyi bir öykü, dikkatli bir fizik muayene ve bilinçli izlem ile PFAPA tanısı kolaylıkla konulabilir. Bu tip olguların erken tanı alması gereksiz antibiyotik kullanımını da önler.

🔖

Cite This Article

HIZARCIOĞLU, M., ASİLSOY, S., KAYSERİLİ, E., GÜLEZ, P., APA, H. (2008). The clinical and laboratory characteristics in patients with periodic fever, apthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Bakırköy Tıp Dergisi, Cilt: 4 - Sayı: 3, 107–110. https://ajindex.com/articles/131515

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (19)

  1. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis and aphthous stomatitis. J Pediatr 1987; 110: 43-46
  2. Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrom in children. J Pediatr 1999; 135: 15-21
  3. Padeh S, Brezniak N, Zemer D, et all. Periodic fever, aphthous stomatitis,pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. J Pediatr 1999; 135: 98-101
  4. Long SS. Syndrome of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA): what it isn’t. What is it? J Pediatr 1999; 135: 1-5
  5. Padeh S. Periodic fever syndromes. Pediatr Clin North Am 2005; 52: 577-609
  6. Marshall GS, Edwards KM, Lawton AR. PFAPA syndrome. Pediatr Infect Dis J 1989; 8: 658-659
  7. Yang KD, Hill HR. Neutrophil function disorders; pathophysiology, prevention, and therapy. J Pediatr 1991; 119: 343-354
  8. Arav-Boger R, Spirer Z. Periodic syndromes of childhood. Adv Pediatr 1997; 44: 389-428
  9. Feder HM jr. Periodic fever, aphthous stomatitis, pharyngitis, adenitis: aclinical review of a new syndrome. Curr Opin Pediatr 2000; 12: 253-256
  10. Meyerhoff J. Familial Mediterranean fever: report of a large family, review of the literature, and discussion of the frequenty of amyloidosis. Medicine 1980; 59: 66-77
  11. Tasher D, Somekh E, Dalal I. PFAPA syndrome-new clinical aspects disclosed. Arch Dis Child 2006; 91: 981-984
  12. Dode C, Cuisset L, Delpech M, Grateau G. TNFRSF1a- associated periodic syndrome (TRAPS) Muckle-Wells syndrome (MWS) and renal amyloidosis. J Nephrol 2003; 16: 435-437
  13. Lee WI, Yang MH, Lee KF, et al. PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) Clin Rheumatol 1999; 18: 207-213
  14. Scholl PR. Periodic fever syndromes. Curr Opin Pediatr 2000; 12: 563-566
  15. Pillet P, Ansoborlo S, Carrere A, Perel Y, Guillard JM. (P)FAPA syndrome: value of cimetidine. Arch Pediatr 2000; 7: 54-57
  16. Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, BitsoriM, Helidonis ES. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002; 86: 434-435
  17. Berlucchı M, Meini A, Plebani A, Bonvini MG, Lombardi D, Nicolai P. Update on treatment of Marshall’s Syndrome (PFAPA syndrome): Report of five cases with review of the literature. Ann Otol Rhinol Laryngol 2003; 112: 365-369
  18. Kurtaran H, Karadağ A, Çatal F, Aktaş D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr 2004; 4: 354-356
  19. Çiftçi E, Özdemir H, İncesoy S,