Cekic S

Bursa Uludağ Üni̇versi̇tesi̇

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Research Topics

Immunodeficiency and Autoimmune Disorders (65) Asthma and respiratory diseases (50) Food Allergy and Anaphylaxis Research (46) Allergic Rhinitis and Sensitization (35) Drug-Induced Adverse Reactions (33)

PFAPA Syndrome Publications

Cluster Analysis in the Differential Diagnosis of PFAPA Syndrome.

Karali Y, Karali Z, Ercan I, Cekic S, Altinok B , et al.
Clinical pediatrics •

The differential diagnosis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome from recurrent upper respiratory tract infections (URTIs) and familial Mediterranean fever (FMF) presents significant clinical challenges due to shared symptomatic presentations. This study aimed to identify diagnostic variables that differentiate PFAPA from URTI and FMF by assessing similarities among cases based on demographic, clinical, and laboratory findings and subsequently forming homogeneous and heterogeneous groups. We included patients diagnosed with PFAPA syndrome, URTI, and acute FMF admitted to the hospital for fever attacks. Demographic data, clinical manifestations, and laboratory results were collected from medical records. Cluster analysis was applied to identify similarities and differences among patients based on these variables. A total of 566 patients (234 with PFAPA, 224 with FMF, 108 with URTI) were analyzed. Cluster analysis, based on demographic, medical history, and clinical/laboratory data, identified 3 distinct clusters: Cluster 1 (n = 231) consisted mostly of URTI patients, Cluster 2 (n = 233) of PFAPA patients, and Cluster 3 (n = 112) of FMF patients, with a similarity level of 0.357. Significant variables distinguishing PFAPA from both FMF and URTI ( < .05) included recurrent fevers before the age of 5 years, the presence of aphthous stomatitis, cervical lymphadenitis, and pharyngitis, an asymptomatic course between fever attacks, metallic taste during attacks, and elevated C-reactive protein and serum amyloid A levels during attacks. This study provides a comprehensive evaluation of PFAPA, using clinical and laboratory characteristics, and complements existing diagnostic criteria for distinguishing PFAPA from conditions commonly confused with it, such as URTI and FMF. The identified differences across demographic, clinical, and laboratory domains provide a strong basis for improving standardized, accurate diagnostic approaches for these disorders.

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Is colchicine more effective to prevent periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis episodes in Mediterranean fever gene variants?

Gunes M, Cekic S, Kilic SS
Pediatrics international : official journal of the Japan Pediatric Society •

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most frequent repetitive fever syndrome in childhood. It is characterized by fever episodes lasting for approximately 3-6 days, once every 3-8 weeks. Clinical and laboratory data for PFAPA syndrome patients between January 2010 and December 2014 followed up at a tertiary pediatric care hospital were reviewed. Four hundred children (256 male, 144 female; mean age at diagnosis, 4.2 ± 2.2 years), were enrolled in the study. During the episodes, mean leukocyte number was high (12 725/mm ) with predominant neutrophils. The mean number of monocytes was 1256/mm , and 90.2% had monocytosis. Serum amyloid A and C-reactive protein were high in 84.6% and in 77.8% of the patients, respectively. Mediterranean fever (MEFV) gene heterozygous mutation was identified in 57 of the 231 patients (24.7%) in whom genetic analysis had been performed. The most frequent mutation was heterozygous M694V (10%, n = 23). Extension of between-episode interval following prophylaxis was noted in 85% of those on regular colchicine treatment (n = 303). In the colchicine group, between-episode interval was prolonged from 18.8 ± 7.9 days (before colchicine treatment) to 49.5 ± 17.6 days on prophylactic colchicine therapy; also, prophylactic treatment was more effective in reducing episode frequency in patients with MEFV gene variant (n = 54, 96%) than in those without (n = 122, 80%; P = 0.003). This study has involved the largest number of PFAPA syndrome patients in the literature. It is particularly important to assess and to demonstrate the high rate of response to colchicine prophylaxis in PFAPA syndrome patients, especially those with MEFV variant. On blood screening, neutrophilia associated with monocytosis and low procalcitonin could contribute to diagnosis.

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