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Periodic fever, aphthosis, pharyngitis, and adenitis (PFAPA) syndrome is a periodic fever syndrome of childhood with an unknown etiology. Our aim was to compare the features between PFAPA syndrome patients from Turkey and those from the US, and patients with and without MEFVvariants, and to test the performance of the Eurofever criteria in excluding other autoinflammatory disorders. Seventy‐one children with PFAPA syndrome, followed in Hacettepe University, in Ankara, Turkey, and 60 patients at Boston Children's Hospital in the US were enrolled. MEFVgene‐variant analysis was performed in 56 patients with Sanger sequencing. In patients from Turkey, symptom onset was at a younger age, fever attacks were of shorter duration, and pharyngitis was more frequent, whereas adenitis, headache, and nausea/vomiting were less frequent during attacks, when compared to patients from the US (P< 0.05). More patients from the Turkish cohort were classified in the familial Mediterranean fever (FMF) group according to the Eurofever criteria than patients from the US (66.2% versus 10%; P< 0.001). Two patients were diagnosed with FMF after MEFVanalysis. Twenty‐one patients (37.5%) had a single MEFVvariant. No significant differences in phenotype were found between patients with and without MEFVvariants. The differences between patients from the Turkish and US cohorts may be due to epigenetic or environmental factors. In addition, the Eurofever FMF criteria may perform better in certain areas, if the weight of ethnic origin parameter or cutoff values were modified.

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever condition in children, with most cases appearing by the age of 5. Although PFAPA is generally a self-limited condition, it can have a major impact on a child’s quality of life, as well as that of their family. Recent research has continued to shed light on the genetic and immunologic factors that play a role in the pathogenesis of PFAPA. There also exists significant heterogeneity in treatment strategies, and progress has been made to develop evidence-based management strategies and establish a standard of care. This review will outline current knowledge regarding the pathogenesis of PFAPA, as well as treatment strategies and our clinical experience.

OBJECTIVE: To review the results of a simple technique of closure of persistent tracheocutaneous fistula (TCF) in children. DESIGN: Retrospective case series. SETTING: Tertiary pediatric otolaryngology referral center. PATIENTS: Children (age, &lt; 18 years) who underwent repair of TCF from July 1, 1991, to August 31, 1996. INTERVENTIONS: Surgical closure of persistent TCF using multilayered closure of de-epithelialized local tissue. Tracheal dissection was not performed. A thermal hemostatic scalpel was used in some cases to assist in de-epithelialization and provide hemostasis without electrocautery near the airway. MAIN OUTCOME MEASURES: Success of closure and number and types of complications. RESULTS: Nine procedures were performed in 8 children. Seven (88%) of 8 primary procedures were successful, but early recurrent TCF developed in 1 patient. Revision surgery using an identical surgical technique, but maintaining endotracheal intubation for 48 hours, was successful in this patient. No complications occurred. CONCLUSIONS: This procedure is a simple, reliable method for closure of TCF in children.Arch Otolaryngol Head Neck Surg. 1997;123:1066-1068.