PFAPA sendromu tanısı alan hastaların klinik ve demografik özellikleri
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Date
2015
Authors
Güneş, Muhammed
Journal Title
Journal ISSN
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Publisher
Uludağ Üniversitesi
Abstract
Periyodik ateş, aftöz stomatit, farenjit ve servikal adenitis (PFAPA) sendromu çocukluk çağında tekrarlayan ateş sendromlarının en sık görülenidir. 3 ila 8 haftada bir olan ortalama 3-6 gün süren ateş ataklarıyla karakterizedir. Atak esnasında bu üç ana belirtiden enaz biri görülür; aftöz stomatit, servikal adenit ve farenjit. Ocak 2010 ve Aralık 2014 arasındaki dönemde PFAPA sendromu tanısı alan toplam olarak 400 hasta (256 erkek, 144 kadın) değerlendirildi. Hastaların tanı esnasında median yaşı 4,22 yıldı. Hastalığın başlangıç median yaşı ise 24,5 ay olarak saptandı. Tek doz intramüsküler 1 mg/kg metil prednizolondan sonra ateşlerinin düştüğü görüldü. Heterozigot MEFV gen mutasyonu 231 hastadan 55 hastada saptandı. Atak esnasında hastaların % 77.8' inde CRP düzeyleri yüksek bulundu. Hastaların % 84.6' sında yüksek SAA düzeyleri saptandı. Prokalsitonin değerleri hastaların % 97.32 ünde negatifti. Ortalama beyaz küre sayısı yüksek bulundu (12724,7 / mm3) ve nötrofil ağırlıklıydı. Ortalama monosit sayısı 1256.16/mm3 bulundu ve hastaların % 74.2' sinde yüksekti. 356 hasta kolşisin ile tedavi edilmiştir. Ataklar arasındaki ortalama süre profilaktik kolşisin tedavisi ile uzadığı görüldü. Dört hastaya tonsillektomi yapıldı. PFAPA sendromu, çocukluk çağında oldukça sık görünmesine rağmen etyolojisi ve patogenezi hala bilinmemektedir. Pediatristler, tekrarlayan ateş atakları ve farenjit bulguları olan bir hasta ile karşılaştıklarında, antibiyotik başlamadan önce PFAPA sendromunu düşünmelidirler. Kolşisin tedavisi ateşli atakların sıklığını azaltmada etkili bulunmuştur. Tonsilektomi sadece seçilmiş hastalara yapılmalıdır.
The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) represents the most common type of recurrent fever in childhood. İt is characterized by episodes of fever lasting for 3–6 days with a recurrence every 3–8 weeks, associated with at least one of the three main signs: aphthous stomatitis, cervical adentitis, and pharyngitis. A total of 400 patients (256 male, 144 female) diagnosed with PFAPA syndrome during the period between January 2010 and December 2014 were evaluated. Patients with PFAPA were included: the median age at the diagnosis was 4,22 years; median age at the onset of disease was 24,59 months. Individual episodes of fever usually resolved with a single intramuscular 1 mg/kg dose of methyl prednisolon. Heterozygous MEFV gene mutations were detected in 57/231 of our patients. At the onset of febril episodes, in 260/334 patients CRP levels were high (77.8%); 148/175 pateints (84.6%) had high SAA levels. Prokalsitonin values were negative in 288/296 patients (97.3%). At the onset of febril episodes, the mean WBC was high (12.724,70/mm3) with a preponderance of neutrophils. The mean monocyte count were 1256,16/mm3, it was high 74.2 % of patients. A total of 356 patients were treated with colchicine. The mean interval between episodes was statistically prolonged in patients who were on prophylactic colchicine therapy. Four patients had tonsillectomy. PFAPA syndrome is a quite common condition in childhood, nevertheless its etiology and pathogenesis are still unknown. Pediatricians have to keep PFAPA Syndrome in their mind when they encounter with a patient with recurrent fever attacks and pharyngitis before starting antibiotics. The colchicine treatment was found effective in decreasing the frequency of fever episodes. Tonsillectomy should be reserved only for selected individuals.
The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) represents the most common type of recurrent fever in childhood. İt is characterized by episodes of fever lasting for 3–6 days with a recurrence every 3–8 weeks, associated with at least one of the three main signs: aphthous stomatitis, cervical adentitis, and pharyngitis. A total of 400 patients (256 male, 144 female) diagnosed with PFAPA syndrome during the period between January 2010 and December 2014 were evaluated. Patients with PFAPA were included: the median age at the diagnosis was 4,22 years; median age at the onset of disease was 24,59 months. Individual episodes of fever usually resolved with a single intramuscular 1 mg/kg dose of methyl prednisolon. Heterozygous MEFV gene mutations were detected in 57/231 of our patients. At the onset of febril episodes, in 260/334 patients CRP levels were high (77.8%); 148/175 pateints (84.6%) had high SAA levels. Prokalsitonin values were negative in 288/296 patients (97.3%). At the onset of febril episodes, the mean WBC was high (12.724,70/mm3) with a preponderance of neutrophils. The mean monocyte count were 1256,16/mm3, it was high 74.2 % of patients. A total of 356 patients were treated with colchicine. The mean interval between episodes was statistically prolonged in patients who were on prophylactic colchicine therapy. Four patients had tonsillectomy. PFAPA syndrome is a quite common condition in childhood, nevertheless its etiology and pathogenesis are still unknown. Pediatricians have to keep PFAPA Syndrome in their mind when they encounter with a patient with recurrent fever attacks and pharyngitis before starting antibiotics. The colchicine treatment was found effective in decreasing the frequency of fever episodes. Tonsillectomy should be reserved only for selected individuals.
Description
Keywords
Periyodik ateş, PFAPA sendromu, Periodic fever, PFAPA syndrome
Citation
Güneş, M. (2015). PFAPA sendromu tanısı alan hastaların klinik ve demografik özellikleri. Yayınlanmamış uzmanlık tezi. Uludağ Üniversitesi Tıp Fakültesi.