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Giriş: Herediter ateş sendromlarından biri olarak sınıflandırılan PFAPA (Periodic fever, apthous stomatitis, pharyngitis and cervical adenitis) sendromu, periyodik ateş, farenjit, servikal lenfadenit ve stomatit ile seyretmektedir. PFAPA sendromu ekartasyon tanısı olup, hastalığın bulguları çocukluk çağında sık görülen bakteriyel tonsillit, viral üst solunum yolu enfeksiyonu ile örtüşmektedir. Hastalığın ataklarına özgül laboratuvar belirteci bulunmamaktadır. Bu çalışmanın amacı tekrarlayan ateş ve tonsillit atakları nedeniyle PFAPA sendromu ön tanısı ile yönlendirilen hastaların izlemde aldıkları tanıları ve PFAPA atağı ile bakteriyel ve viral tonsillit arasında laboratuvar değerlerindeki farkları ortaya koymaktır. Metod: Bu çalışmaya PFAPA şüphesi ile yönlendirilen toplam 69 çocuk dahil edilmiştir. Bu hastalar en az 2 atak sırasında tek bir hekim tarafından değerlendirilerek, PFAPA sendromu tanısı almıştır. Ateş atakları düzenli aralıklarla 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 A beta hemolitik streptokok geçiren 9 hasta ve viral tonsillit geçiren 15 hasta ile karşılaştırılmıştır. Sonuçlar: Tekrarlayan tonsillofarenjit tanısı alan 23 hastadan ikisi mutasyon analizi ile ailevi Akdeniz ateşi (AAA), biri gastroözafajiyel reflü tanısı almış kalan 20 hastanın ise 15’inin takiplerinde ateşi hiç olmamış, beşinin ise tanı kriterlerinde belirtildiği gibi düzenli aralıklarla tekrarlayan ateşleri gözlenmemiştir. İzlemde PFAPA tanısı alan 46 hasta ile tekrarlayan tonsillit tanısı alanlar karşılaştırıldığında, PFAPA tanısı alan hastaların ailelerinde istatistiksel anlamlı olarak daha fazla tekrarlayan 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 ile karşılaştırıldığında prokalsitonin seviyelerinde anlamlı fark saptanmazken (p=0.053), PFAPA atağında sedimentasyon daha yüksek, lökosit sayısı daha düşük saptandı (p değerleri 0.021 ve <0,01). Tartışma: Beş yaşın altında tekrarlayan ateş ve farenjit nedeniyle başvuran hastalarda, atak özelliklerinin ve atak aralıklarının değerlendirilmesi PFAPA tanısının doğru konulması açısından önemlidir. Hastaların aile hikayesi, tekrarlayan farenjit ve ailevi Akdeniz ateşi açısından mutlaka sorgulanmalıdır. Prokalsitonin seviyelerinde PFAPA atağı ve bakteriyel tonsillit arasında farklılık saptanmamıştır.
PFAPA (Periodic fever, apthous stomatitis, pharyngitis and cervical adenitis), which is classified as one of the hereditary fever syndrome, is seen with periodic fever, farenitis, cervical lymphadenitis and stomatitis. PFAPA syndrome is a diagnosis of ecartation, and the symptoms of the disease are combined with bacterial tonsillitis, viral upper respiratory infection, which is common in childhood. There is no specific laboratory indicator for the disease attacks. The aim of this study is to reveal the diagnoses that patients guided by pre-diagnosis of PFAPA syndrome due to repeated fever and tonsillitis attacks and the differences in laboratory values between PFAPA attacks and bacterial and viral tonsillitis. Method: The study included a total of 69 children directed with PFAPA suspicion. This disease was diagnosed with PFAPA syndrome, assessed by a single physician during the 2 attacks. Patients who do not repeat inflammatory attacks on regular intervals and have other symptoms of disease have been considered to be repeated tonsillopharenitis. The laboratory values of PFAPA patients (lococyte, erythrosite sedimentation rate, C reactive protein and procalcitonin) were compared with 9 patients who suffered beta hemolithic streptococcus in Group A and 15 patients who suffered viral tonsillitis. Results: Two of 23 patients diagnosed with repeated tonsillopharenitis with mutation analysis with family Mediterranean fever (AAA), one diagnosed with gastro-efacial reflux; the remaining 20 patients have never been diagnosed with fever in the follow-up, and the fifth have not been observed with regular intervals of repeated fever as indicated in the diagnostic criteria. Compared to those diagnosed with recurring tonsillitis with 46 patients diagnosed with PFAPA in the survey, the family of patients diagnosed with PFAPA had statistically significantly more recurring tonsillitis history (60.9% (p=0.041) and their initial age was smaller (p=0.022). Compared with patients with PFAPA and group A streptococcus tonsillitis, there is no significant difference in procalcitonin levels (p=0. 053), higher sedimentation in the PFAPA attack, lower leukocytes were found (p values 0.021 and <0,01). Discussions: In patients who apply for repeated fever and farenitis under the age of five, the assessment of the attacks characteristics and intervals of attacks is important for the correct diagnosis of PFAPA. The family history of the patients, repeated pharenitis and family Mediterranean fire should be questioned. There is no difference in procalcitonin levels between PFAPA attack and bacterial tonsillitis.
Introduction: 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. 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 charactersitic physical findings were diagnosed as PFAPA syndrome. Others who do not have regular fever were diagnosed as recurrrent tonsillopharngitis. Leucocyte and acute phase reactants (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 more patients 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.
Alan : Sağlık Bilimleri
Dergi Türü : Uluslararası
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