Burçin NALBANTOĞLU, Metin UYSALOL, Erkut KARASU, Burcu ÖZDİLEK

İki Vaka Nedeniyle PFAPA Sendromu

Yineleyen veya periyodik ateş yakınmasına çocuk hekimli- ğinde nispeten sık rastlanmaktadır. PFAPA sendromu, yük- sek ateş, servikal lenfadenopati, aftöz stomatit ve farenjit ile karakterizedir. Sekiz yaşında kız hasta olan ilk vakanın 3-4 haftada bir yineleyen ateş, farenjit ve aftöz stomatit atakları vardı. Ateşli dönem 5-6 gün sürmekte antibiyotik tedavisine yanıt alınamamaktaydı. Atak sırasında akut faz reaktanlarının yükseldiği ve ataklar arasında tamamen normale döndüğü saptandı. Bu bulgularla hastada PFAPA sendromu düşünüldü. Hastanın yakınmaları tek doz predni- zon ile dramatik bir şekilde düzeldi. Üç buçuk yaşında erkek hasta olan ikinci vakanın ayda bir membranöz tonsil- lit, yüksek ateş yakınmaları vardı. Atakların bazılarında servikal lenfadenopati gözlenirken, aftöz stomatit hiç olmu- yordu. Ataklar sırasında antibiyotik kullanımına rağmen, ateş 5-6 gün sürüyor ve akut faz reaktanları yüksek olarak bulunuyordu. Atak sırasında tek doz prednizon tedavisine yanıt veren hastaya PFAPA tanısı kondu. PFAPA sendromu- nun 5 yaş üzeri de görülebileceğini, aftöz stomatit olmadan da tanı koyulabileceğini vurgulamak ve iki atipik vaka nedeniyle PFAPA sendromunu vurgulamak ve literatürü derlemek istedik

Anahtar Kelimeler:

PFAPA, tekrarlayan ateş, çocuklar

Two Cases with PFAPA Syndrome

Recurrent or periodic fever is seen frequently in pediatric medicine. Diagnostic criteria of PFAPA syndrome is cha- racterized by fever, cervical lymphadenopathy, aphthous stomatitis and pharyngitis. First case was a 8 year-old girl and she had recurrent attacks of fever, pharyngitis and aphthous stomatitis in every 3-4 weeks. Fever lasted 5-6 days and no response to antibiotics was received. Acute phase reactants elevated during the attacks and returned to normal between attacks. Symptoms of the patient dramati- cally returned to normal after single dose of prednisone. Second case was 3.5 year-old boy and he had recurrent attacks of membranous tonsillitis and high fever once a month. Cervical lymphadenopathy was observed in some of the attacks but without any aphthous stomatitis. Fever las- ted 5-6 days despite antibiotic use and acute phase reac- tants elevated. Symptoms of the patient returned to normal after a single dose of prednisone. We want both to empha- size PFAPA syndrome with two atypic patients and also review the literature

Keywords:

PFAPA, recurrent fever, children,

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