The rs13075270 and rs13092160 polymorphisms of CCR1 and CCR3 genes on oral aphthous-like lesions in PFAPA syndrome

Fever is a common symptom of infection in children. Periodic fever syndromes are less common but more complex. One of these Periodic fever syndromes is PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome which is known as the most benign syndromes. The cause of this disease is unknown. Various factors, including environmental and genetic factors, are involved in the development of this disease. In this study, the association of rs13075270 and rs13092160 polymorphisms were investigated in CCR1 and CCR3 genes with susceptibility to this syndrome in the Chinese population. In this regard, 38 patients with PFAPA syndrome and 100 healthy individuals were selected. After DNA sampling and extraction, polymorphisms of CCR1 and CCR3 receptor genes were examined by the PCR-RFLP method. Findings were analyzed using SPSS software version 22 with a significant level of P <0.05. The frequency of T/T genotype rs13092160 polymorphism in the patient and control groups was 78.95% and 83%, respectively, C/T genotype was 21.05% and 17% (P = 0.421). The frequency of the C/C genotype was 0 in both groups. Regarding rs13075270 polymorphism, the frequency of T/T genotype in patient and control groups was 15.79% and 81%, C/T genotype was 78.95% and 18% and C/C genotype was 5.26% and 1%, respectively (P<0.05). Thus, in rs13075270 polymorphism, the C/T genotype was associated with the risk of PFAPA syndrome (P<0.05), but rs13092160 polymorphism did not show a significant difference between individuals with PFAPA syndrome and controls.

The High Necessity of Rapid Antigen Detection Test (RADT) use to Guide Antibiotic Prescribing Pattern for Acute Pharyngotonsillitis in Syria

The objectives of this study were to assess the percentage of the inappropriate antibiotics prescribing for clinically diagnosed Group A β-hemolytic streptococcus (GABHS) caused acute pharyngotonsillitis (APT), and to investigate factors influencing this prescribing and the possibility and necessity of routine usage of RADT for rational treatment of APT in different primary care facilities in Latakia, Syria. Throat swabs were collected from 80 patients, ≥ 5 years old, seen in urban or rural pharmacies, otolaryngology clinic or emergency unit of University Tishreen hospital over a period of 6 months, who were diagnosed and treated for APT depending on clinical findings. RADT was applied and McIsaac score (MIS) was recorded for all patients. Throat cultures were done only for negative RADT cases seen in the hospital. The chi square (χ2) statistical test was used for comparing categorical variables. A P value of <0.05 was considered significant. Antibiotics were prescribed for 59 patients (74%) among them RADT results were positive for only 9 patients (15.25%) with a percentage of inappropriate antibiotic prescribing of (84.75%). Cultures were GABHS negative for all negative RADT cases. The most frequently prescribed antibiotic was azithromycin (50.8%). Antibiotics prescribing was significantly influenced by the presence of fever (P=0.041), anterior cervical adenitis (P=0.0003), and MIS≤0 (P=0.0001). We found a significance association of the presence of tonsillar exudate (P=0.013) and MIS≥4 (P=0.002) with positive RADT results. There was no significant difference in antibiotic prescribing according to age, sex or place of consultation. Our findings highlight the great need to use RADT in clinical practice as important adjuvant tool in APT diagnosis to reduce the percentage of antibiotic prescribing and so limiting of bacterial resistance.

POS1294 EFFECT OF EMOTIONAL TRIGGERS ON THE FREQUENCY OF PFAPA ATTACKS - A MULTICENTER STUDY

Background:The fact that mental state may serve as a trigger for attacks of periodic fever diseases is known in Familial Mediterranean fever (FMF). There is a lack of information regarding the role of emotional triggers for PFAPA attacks.Objectives:To examine whether emotional distress may trigger PFAPA attacks.Methods:Enrolled were patients with active PFAPA, from two Israeli medical centers. Researchers contacted their parents by phone in two occasions: a. within the two weeks of returning to school after the first COCID-19 lockdown; b. during the summer vacation (i.e., non-stressful period). Parents were asked regarding the occurrence of PFAPA attacks within the two weeks preceding the calls. The relative stress levels of the two periods were validated by an emotional distress scale questionnaire. Differences between occurrences of attacks during these two periods of time were recorded.Results:One-hundred and six pediatric patients enrolled in the study. Mean age was 7.37± 2.9. In the stressful period, 41 (38.7%) reported at least one attack during the preceding 2 weeks, compared to 24 (22.6%) in the non-stressful period (p = 0.017). Scores for the mean emotional distress questionnaire were higher in the stressful period compared to the non-stressful period (35.6± 8.1 vs. 32.1± 7.7, respectively, P = 0.047).Conclusion:This study shows that during a period of stress for children there were more PFAPA attacks compared to a relaxed period. These findings indicate that mental factors such as stress or excitement may be triggers for PFAPA attacks.Table 1.Attacks status during prior 2 weeks during stressful or non-stressful eventOutcomeFirst call(May 2020)Second call (August 2020)P-valuePediatric emotional distress score (PEDS), mean (±SD)35.6 (±8.1)32.1 (±7.7)0.047Any attacks during last 2 week, n (%)41 (39.8%)24 (24.2%)0.017Number of attacks0.04 One, n (%)27 (25.5%)19 (17.9%) Two, n (%)14 (13.2%)5 (4.7%)References:[1]Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110(1):43-46. doi:10.1016/S0022-3476(87)80285-8[2]Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr Int J Paediatr. 2010;99(2):178-184. doi:10.1111/j.1651-2227.2009.01554.x[3]Hall, J, Lindorff A. Children’s Transition to School: Relationships Between Preschool Attendance, Cortisol Patterns, and Effortful Control. The Educational and Developmental Psychologist. Educ Dev Psychol. 2017;34(1):1-18[4]Stokes SJ, Saylor CF, Swenson CC, Daugherty TK. A comparison of children’s behaviors following three types of stressors. Child Psychiatry Hum Dev. 1995;26(2):113-123. doi:10.1007/BF02353235[5]Førsvoll J, Kristoffersen EK, Øymar K. The immunology of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; what can the tonsils reveal. A literature review. Int J Pediatr Otorhinolaryngol. 2020;130. doi:10.1016/j.ijporl.2019.109795[6]Gidron Y, Berkovitch M, Press J. Psychosocial correlates of incidence of attacks in children with Familial Mediterranean Fever. J Behav Med. 2003;26(2):95-104. doi:10.1023/A:1023038504481[7]Makay B, Emiroğlu N, Ünsal E. Depression and anxiety in children and adolescents with familial Mediterranean fever. Clin Rheumatol. 2010;29(4):375-379. doi:10.1007/s10067-009-1330-9[8]Kraszewska-Głomba B, Matkowska-Kocjan A, Szenborn L. The Pathogenesis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Review of Current Research. Mediators Inflamm 2015;2015:563876. doi:10.1155/2015/563876Disclosure of Interests:None declared

Management of Fever Attack in a Patient with PFAPA Syndrome

Fever is a common symptom in childhood and primarily suggests infectious diseases. The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is a periodic fever syndrome of unknown incidence and is not rare in childhood. In this case report, the management of a patient who was followed up for PFAPA syndrome during a fever attack is presented.

Effectiveness of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: a case series

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever (febrile attacks) lasting for 3–6 days and recurring regularly. In this syndrome, fever is associated with aphthous stomatitis, pharyngitis, and cervical adenitis.1–4 Almost all patients with PFAPA syndrome spontaneously resolve approximately 8 years after the onset of symptoms. However, the cause and etiology of PFAPA syndrome remain unclear.4 Oral prednisolone reduces fever during febrile attacks.5–7 Tonsillectomy has also been reported to induce remission of PFAPA syndrome.8–12 However, tonsillectomy is not a popular treatment for PFAPA syndrome because its effectiveness is still unclear. Furthermore, tonsillectomy is generally recommended for patients aged 3 years and older to avoid post-operative complications.13 We report here on four patients aged 3 years and older with PFAPA syndrome. Tonsillectomy completely eliminated febrile attacks and was safely performed without post-operative complications in all of our patients.

Una PFAPA che diventa un Behçet

PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common periodic fever syndrome in children. The disease appears to cluster in families, but the pathogenesis is unknown. The paper reports the description of a clinical case of a 12-year-old girl diagnosed with PFAPA syndrome from the age of one and half years. The exacerbations did not decrease with growth as it usually happens, but genital ulcers appeared associated with HLA-B51 positivity, orienting the diagnosis towards Behçet’s spectrum disorders.