Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (pfapa) syndrome in children.
BACKGROUND
Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome refers to a group of primary immunodeficiencies, namely autoinflammatory diseases. Most pediatricians and otolaryngologists do not suspect PFAPA syndrome when treating recurrent pharyngitis (according to Ukrainian classification - tonsillitis) and stomatitis. Therefore, patients with a given syndrome receive unnecessary treatment (antibiotic therapy or antiviral drugs) and the diagnosis is made late. The aim of the research was to provide pediatricians, family physicians and otolaryngologists with information on the importance of early diagnosis of PFAPA syndrome.


METHODS
The analysis of the prevalence and diagnosis of PFAPA syndrome in Ukraine and worldwide has been made as well as a late diagnosis of PFAPA syndrome in a child living in Ivano-Frankivsk, Ukraine has been described (case report).


RESULTS
The Сase report 7-year-old boy, who grows and develops normally. The symptoms of pharyngitis including high body temperature (>40 º С), sore throat and white spots on the tonsils appeared for the first time at the age of two years. The boy received antibacterial drugs about 10 times a year. During a four-year period of recurrent episodes of the disease antimicrobial susceptibility testing to determine susceptibility of the oropharyngeal flora to the antibiotics were continuously performed, different blood tests for herpes viruses, Epstein-Barr virus infection and cytomegalovirus in particular were made using the enzyme immunoassay (EIA) and polymerase chain reaction (PCR) in addition to long-term treatment.


CONCLUSIONS
An example of late diagnosing PFAPA syndrome (four years after the onset of first symptoms) resulting in regular examinations, medical manoeuvres, outpatient and inpatient treatment, use of antibiotic therapy including intravenous injections on a monthly basis has been studied.
State Higher Educational Institution "Ivano-Frankivsk National Medical University", Department Of Children Diseases Of Postgraduate Medical Education Faculty, Ivano-Frankivsk, Ukraine.
Vira B
VB
Semianchuk
D002648:Child; D057210:Delayed Diagnosis; D005334:Fever / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008199:Lymphadenitis / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D010612:Pharyngitis / Q000175:diagnosis* / Q000188:drug therapy; D013281:Stomatitis, Aphthous / Q000175:diagnosis* / Q000188:drug therapy; D013577:Syndrome; D014455:Ukraine
 aphthous stomatitis;  cervical adenitis ;  pharyngitis; periodic fever