Assessment of sleep in children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is the most common cause of periodic fever in childhood. This study aimed to investigate sleep patterns and possible factors that are associated with sleep disturbances among children with PFAPA syndrome. METHODS: Sixty-two patients with PFAPA and 68 age and sex matched healthy controls were enrolled in the study. Patients who had an attack during the former 2 weeks were not included. Demographic and anthropometric data, duration of fever episodes, laboratory results, and clinical manifestations of patients were recorded. The Children`s Sleep Habits Questionnaire was administered. RESULTS: The total sleep scores of patients with PFAPA were significantly higher than the control group (49.6 ± 10.7 vs. 38.3 ± 7.5, p = 0.002 ). Children with PFAPA had significantly higher scores regarding sleep-onset delay, sleep anxiety and night wakening (p=0.003, p=0.007, and p=0.014, respectively). Total sleep durations were similar between children with PFAPA and the control group. There was a significant positive correlation between the total sleep score and disease duration (r=0.425, p=0.002). Also there was a significant positive correlation between disease duration and sleep onset delay (r=0.561, p < 0.001) and night wakening (r=0.327, p=0.003). CONCLUSION: This study showed for the first time that patients with PFAPA have significantly disturbed sleep when compared to otherwise normal children. This study emphasized the need to assess sleep problems in children with PFAPA.

Procalcitonin and C-reactive protein-based decision tree model for distinguishing PFAPA flares from acute infections.

As no specific laboratory test has been identified, PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) remains a diagnosis of exclusion. We searched for a practical use of procalcitonin (PCT) and C-reactive protein (CRP) in distinguishing PFAPA attacks from acute bacterial and viral infections. Levels of PCT and CRP were measured in 38 patients with PFAPA and 81 children diagnosed with an acute bacterial (n=42) or viral (n=39) infection. Statistical analysis with the use of the C4.5 algorithm resulted in the following decision tree: viral infection if CRP≤19.1 mg/L; otherwise for cases with CRP>19.1 mg/L: bacterial infection if PCT>0.65ng/mL, PFAPA if PCT≤0.65 ng/mL. The model was tested using a 10-fold cross validation and in an independent test cohort (n=30), the rule's overall accuracy was 76.4% and 90% respectively. Although limited by a small sample size, the obtained decision tree might present a potential diagnostic tool for distinguishing PFAPA flares from acute infections when interpreted cautiously and with reference to the clinical context.