PFAPA Sendromu, Tekrarlayan Tonsillofarenjit ve Enfeksiyöz Tonsillofarenjit Ayrımında Klinik ve Laboratuvar Özellikler

Amaç: Kalıtsal ateş sendromlarından biri olarak sınıflandırılan PFAPA (Periodic fever, apthous stomatitis, pharyngitis andcervical adenitis) sendromu, periyodik ateş, farenjit, servikal lenfadenit ve stomatit ile seyretmektedir. PFAPA sendromudışlama tanısı olup, hastalığın bulguları çocukluk çağında sık görülen bakteriyel tonsillit, viral üst solunum yolu enfeksiyonuile örtüşmektedir. Hastalığın ataklarına özgül laboratuvar belirteci bulunmamaktadır. Bu çalışmanın amacı tekrarlayanateş ve tonsillit atakları nedeniyle PFAPA sendromu ön tanısı ile yönlendirilen hastaların izlemde aldıkları tanıları sunmakve PFAPA atağı geçiren çocuklarla ile bakteriyel veya viral tonsillit geçiren çocuklarınlaboratuvar değerlerindeki farklarıortaya koymaktır.Gereç ve Yöntemler: Bu çalışmaya PFAPA şüphesi ile yönlendirilen toplam 69 çocuk dahil edilmiştir. Bu hastalar enaz 2 atak sırasında tek bir hekim tarafından değerlendirilerek, PFAPA sendromu tanısı almıştır. Ateş atakları düzenliaralıklarla tekrarlamayan ve diğer hastalık bulguları olan hastalar tekrarlayan tonsillofarenjit olarak değerlendirilmiştir.PFAPA hastalarının laboratuvar değerleri (lökosit, eritrosit sedimentasyon hızı, C reaktif protein ve prokalsitonin), Grup Abeta hemolitik streptokok geçiren 9 hasta ve viral tonsillit geçiren 15 hasta ile karşılaştırılmıştır.Bulgular: Tekrarlayan tonsillofarenjit tanısı alan 23 hastadan ikisi mutasyon analizi ile ailevi Akdeniz ateşi (AAA), birigastroözafajiyel reflü tanısı almış; kalan 20 hastanın ise 15’inin takiplerinde ateşi hiç olmamış, beşinin ise tanı kriterlerindebelirtildiği gibi düzenli aralıklarla tekrarlayan ateşleri gözlenmemiştir. İzlemde PFAPA tanısı alan 46 hasta ile tekrarlayantonsillit tanısı alanlar karşılaştırıldığında, PFAPA tanısı alan hastaların ailelerinde istatistiksel anlamlı olarak daha fazlatekrarlayan tonsillit hikayesi mevcuttu (%60,9) (p=0.041) ve yakınmalarının başlangıç yaşı daha küçüktü (p=0.022).PFAPA hastaları ile Grup A streptokok tonsilliti geçiren hastalar karşılaştırıldığında, prokalsitonin seviyelerinde anlamlıfark saptanmazken (p=0.053), PFAPA atağında sedimentasyon daha yüksek, lökosit sayısı daha düşük saptandı (pdeğerleri 0.021 ve

Objective: PFAPA (Periodic fever, apthous stomatitis, pharyngitis and cervical adenitis) is characterized by recurrent periodic fever, pharnygitis, stomatitis and classified under the hereditary fever syndromes. Diagnosis is based on exclusion of diseases as bacterial and viral pharngitis commonly seen during childhood. There is no specific laboratory test during attacks. In this study, we recruited patients with complaints of recurrent fever and pharngitis and evaluated their final diagnosis. Also we aimed to compare the laboratory findings of PFAPA patients with bacterial and viral tonsillits. Material and Methods: Sixty-nine patients with recurrent fever and pharngitis were included in this study. All patients were evaluated by the same physician. Patients who had regular fever attacks and characteristic physical findings were diagnosed as PFAPA syndrome. Others who do not have regular fever were diagnosed as recurrrent tonsillopharngitis. Leucocyte and acute phase reactant (erytrocyte sedimentation rate, C reactive protein, procalcitonin) levels of patients with recurrent fever were compared with patients with group A streptococcus tonsillitis (n=9) and viral tonsillitis (n=15). Results: On follow-up 46 patients were diagnosed as PFAPA syndrome and 23 patients were diagnosed as recurrent tonsillopharngitis. Among patients with recurrent tonsillopharngitis, two patients were diagnosed as familial Mediterrenean fever (FMF), one was diagnosed as gastroesophageal reflux (GER) on follow-up. Remaining 20 patients had neither fever on follow-up or had irregular fever. When we compared patients with PFAPA syndrome and recurrent tonsillipharngitis, patients with PFAPA syndrome had statistically higher percent of family member with familiy history of recurrent tonsillitis (%60.9) (p=0.041) and were younger at disease onset (p=0.022). The procalcitonin levels of PFAPA patients were indifferent compared to bacterial tonsillitis patients (p=0.053). Conclusion: Patients who admitted for recurrent fever and tonsillitis before 5 years of age should be followed for attack characteristics for correct diagnosis of PFAPA syndrome. Family history should be questioned for recurrent tonsillitis and familial mediterrenean fever. Procalcitonin levels were indifferent between PFAPA patients and bacterial tonsillitis during attacks.

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