İki Vaka Nedeniyle PFAPA Sendromu

Year 2011, , 177 - 180, 01.10.2011

Burçin Nalbantoğlu Metin Uysalol Erkut Karasu Burcu Özdilek

https://doi.org/10.5222/j.child.2011.177

Abstract

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

Keywords

PFAPA, tekrarlayan ateş, çocuklar

Two Cases with PFAPA Syndrome

Abstract

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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