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Objectives: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever syndrome during early childhood period with regular febrile attacks of sterile upper airway inflammation. The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily. The aim of this study is to explore the immunological basis of PFAPA by evaluating the cellular properties of tonsils, and microbial exposition such as Helicobacter pylori on tonsillectomy materials. Methods: The paraffinized tonsil samples of 26 PFAPA and 29 control patients with obstructive upper airway disorder were compared in terms of immunohistochemical staining features including CD4, CD8, CD123, CD1a, CD20, and H. pylori. Results: The median number of CD8+ cells was 1485 (1218-1287) in PFAPA while it was 1003 (852–1261.5) in control group and the difference was statistically significant (p=0.001). Similarly, CD4+ cell counts were statistically higher in PFAPA group than control (833.5 vs 622). The ratio of CD4/CD8 did not differ between two groups; also, there was no statistically difference in terms of the other immunohistochemical staining results, such as CD20, CD1a, CD123 and H. pylori. Conclusion: This is the largest number of pediatric tonsillar tissue study of PFAPA patients in current literature and we emphasized the triggering effects of CD8+ and CD4+ T-cells on PFAPA tonsils. Key Points:• The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily.• In current study, 92.3% of our patients did not experience any attacks following operation similarly with literature.• We observed the increased number of CD4+ and CD8+ T cell counts on PFAPA tonsils compared to control group and emphasized the active role of both CD4+ and CD8+ cells localized on PFAPA tonsils in immune dysregulation.• Some other cell types evaluated in this study such as CD19+ (B cells), CD1a (dendritic cells), and CD123 (IL-3 receptors, for pluripotent stem cells) and H. pylori did not differ in PFAPA patients compared to the control group.

Objectives: This study aimed to evaluate the risk of tuberculosis (TB) disease in children receiving biological agents for rheumatologic diseases, focusing on appropriate screening tests in a high-priority country for TB control. Patients and methods: One hundred nine children (56 females, 53 males; range, 3.4 to 16.2 years) who received any biological agent for rheumatologic diseases for more than two years between May 2012 and October 2021 were included in this retrospective study. Patients were screened for TB infection using tuberculin skin test (TST) or interferon-gamma release assay (IGRA). Following the initial evaluation, patients were clinically examined for TB every three months by a comprehensive medical history and physical examination, and every 12 months using TST or IGRA. Results: At the initiation of the biological agent, the patients’ mean age was 12.4±4.5 years. The average follow-up duration was 3.6±1.3 years (range, 2.6 to 10.2 years) for patients treated with biological agents. Each patient had a documented Bacillus Calmette-Guérin vaccination. Before the initiating of therapy, TST was performed alone in 45 (41.3%) patients and in combination with IGRA in 64 (58.7%) patients. In the 64 patients who underwent both TST and IGRA, IGRA revealed nine (14.1%) positive results. Six (66.7%) of these nine patients, however, had negative baseline TST. Four (7.3%) of the 55 individuals whose initial IGRA results were negative also had positive TST results. Overall, no TB disease was observed after a follow-up period. Conclusion: This study reveals that biological agents were not associated with an increased risk of TB disease in closely monitored children. Additionally, the concomitant use of TST and IGRA for screening of TB is reasonable in patients receiving biological agents.

Comparing the wrist joint position sense and hand functions between children with juvenile idiopathic arthritis (JIA) and healthy controls, and determining possible relationships between these parameters in children with JIA were the aims of this study. Twenty children with polyarticular JIA with wrist involvement (JIAWrist+), 20 children with other subtypes of JIA without wrist involvement (JIAWrist−), and 20 healthy controls were included. Wrist joint position sense was evaluated by measuring joint repositioning error. Hand functions were assessed by using the Purdue Pegboard test, hand grip strength, pinch strength, and Duruoz Hand Index. Joint position sense and hand functions were diminished in the JIAWrist+ group compared with healthy control group ( P < .05). Few moderate relationships were detected between hand functions and wrist joint position sense ( P < .05). Improving proprioceptive acuity by appropriate training methods may have a role in enhancing hand functions.

Objectives: This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis. Patients and methods: Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.5 to 21 years) who were diagnosed with chronic non-bacterial osteomyelitis in three pediatric rheumatology centers were retrospectively analyzed. The demographic, clinical, and laboratory findings of the patients were recorded. Results: The median age at the time of diagnosis was 10.2 years. The median time from symptom onset to diagnosis was 6.5 months. The median follow-up was 18.5 months. The most frequent symptom at onset was arthralgia (75.0%). The most frequently involved bone was the femur (67.9%). Eight (63.6%) of 12 patients had at least one Mediterranean fever gene (MEFV) mutation, indicating a significantly higher prevalence than the Turkish population (14.8%). Five of these patients fulfilled the diagnostic criteria for familial Mediterranean fever (FMF). All patients received non-steroidal anti-inflammatory drugs. Other drugs were methotrexate (46.4%), sulfasalazine (39%), corticosteroids (25%), anti-tumor necrosis factor (anti-TNF) agents (32%), pamidronate (25%), and colchicine (21.4%). Six of eight patients with MEFV mutations were administered with colchicine, and all of them responded to treatment. Conclusion: Clinical evolution and imaging investigations should be carefully performed to prevent any delay in the diagnosis of patients with chronic non-bacterial osteomyelitis. Based on our study results, FMF coexistence is worth investigating in patients with chronic non-bacterial osteomyelitis, particularly in the Turkish population.

El diagnóstico precoz y el tratamiento de la artritis son fundamentales para el pronóstico de la enfermedad. Especialmente durante la fase activa de la artritis idiopática juvenil (AIJ), es necesario un diagnóstico rápido para manejar la enfermedad adecuadamente. Se han investigado nuevos marcadores inflamatorios como la proporción de neutrófilos a linfocitos (NLR) y la proporción de monocitos a linfocitos (MLR), el volumen plaquetario medio (MPV) y en la amplitud de distribución plaquetaria (PDW) en varios trastornos inflamatorios.Objetivo: determinar el valor diagnóstico de NLR, MLR, MPV y PDW para diferenciar la AIJ en niños con artritis.Pacientes y Método: Estudio de casos y controles con 324 niños con artritis (casos) y 324 niños sanos (controles). Los niños con artritis se agruparon en AIJ y no AIJ. Se revisaron retrospectivamente las historias clínicas de niños de 0 a 18 años. Los parámetros hematológicos se registraron al momento del diagnóstico. Se analizaron la NLR, MLR, MPV y PDW en los grupos en estudio.Resultados: En el grupo de casos, el 52,8% fueron niños y el 47,2% niñas; la edad media fue de 7,7 ± 4,0 años. La NLR en el grupo de casos fue significativamente mayor que en el grupo control (p=0,001). La media del MPV fue menor en el grupo de casos que en el grupo de control (p=0,001). No hubo diferencias en NLR y MPV entre los grupos AIJ y no AIJ (p=0,062, p=0,689). El PDW medio del grupo AIJ fue menor que el del grupo sin AIJ (p=0,001).Conclusión: El aumento de NLR puede indicar inflamación, pero no tiene superioridad para distinguir la AIJ de otras causas de artritis. El ancho de distribución de las plaquetas fue menor en los pacientes con AIJ, pero su utilidad clínica es limitada.

Background: Little is known about the long-term efficacy and safety of canakinumab in paediatric FMF patients. Aim: To present the single centre experience of colchicine-resistant paediatric-onset FMF patients who were treated with canakinumab by off-label use since 2012. Methods: The hospital files of 15 children who used canakinumab were retrospectively evaluated. Clinical and laboratory data of each visit were recorded. Drug-related adverse events were recorded. Complete remission was described as no attacks and normal acute phase reactants; partial remission was defined as decrease in severity and rate of attacks and/or elevated acute phase reactants with anti-IL-1 treatment. Results: The average duration of canakinumab use was 23.9 months (min:12, max:58 months). Twelve patients were M694V homozygotes. Eleven patients achieved complete remission after the first dose at 2 months and 12 patients at 6 months. Canakinumab interval was shortened in 2 patients from 150 mg/8 weeks to 150 mg/4 weeks. Except one, 14 patients achieved complete remission by 12 months. Two patients had mild urinary tract infections. One patient had bronchopneumonia requiring hospitalization. Two patients had teeth abscess. There were no serious adverse events such as opportunistic infections, malignancies, or deaths. Besides, no significant laboratory abnormalities occurred in complete blood count parameters, liver and kidney function tests. Conclusion: To the best of our knowledge, this is the longest outcome study about canakinumab use in paediatric FMF patients. This study suggested that canakinumab is safe and effective in children with FMF in the long term.

Background: Colchicine is an anti-inflammatory agent used for preventing familial Mediterranean fever (FMF) attacks and amyloidosis. A significant number of patients are non-responsive or intolerant to the domestic drug colchicum dispert. Aims: To compare the efficacy and side effects of colchicum dispert and colchicine opocalcium in children with FMF. Study Design: A total of 29 children with FMF who used colchicum dispert for at least 6 months initially and colchicine opocalcium for another consecutive 6 months were included. Sex and gender equity in research was considered. Clinical features, visual analog scale for pain scores, exercise-induced leg pain, and FMF severity scores with laboratory parameters were evaluated for both the treatment periods. Bristol stool chart and number of stools per 24 hours were recorded to compare the gastrointestinal side effects. Methods: A total of 29 children with FMF who used colchicum dispert for at least 6 months initially and colchicine opocalcium for another consecutive 6 months were included. Sex and gender equity in research was considered. Clinical features, visual analog scale for pain scores, exercise-induced leg pain, and FMF severity scores with laboratory parameters were evaluated for both the treatment periods. Bristol stool chart and number of stools per 24 hours were recorded to compare the gastrointestinal side effects. Results: The major indication was non-responsiveness in 18 patients (62%) and intolerance in 11 patients (38%). Usage of colchicine opocalcium (significantly higher dosage than colchicum dispert) showed statistically significant beneficial effects on the number and duration of attacks, visual analog scale for pain, exercise-induced leg pain scores, and FMF severity scores (p

The aim of this study is to evaluate our patients with the newly developed international severity score for FMF (ISSF) and make comparisons with the literature.This cross-sectional study included patients with FMF, were between 6 months and 18 years old, and were using colchicine/colchicine+IL-1 inhibitor for at least 6 months. The patients were classified as mild, intermediate, and severe based on their scores between 1 and 10. In addition to scoring, those who have additional pathological or silent mutation were compared based on these classifications.Our patients consist of 88 women 72 men total 160. The mean age, age of onset, and age of diagnosis were 12 ± 4.3, 4.9 ± 3.7, and 7.5 ± 4 years, respectively, and the mean diagnostic delay was 2.6 ± 3years. When our large patient population is evaluated with ISSF, the disease severity is mostly intermediate. According to ISSF, mild, intermediate, and severe diseases were 21.3%, 70.5%, and 8.1%, respectively. The duration of attacks shorten with increasing age (p0.05), and there is an increase in the involvement of other organs during the attacks among patients older than 7 years.ISSF appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients. Key Points • Our article is the first study to evaluate ISSF performance in the pediatric population. • International severity score for FMF (ISSF) appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients. • We think that the addition of pathogenic mutations and inheritance model to the ISSF scoring system as important determinants of disease severity, especially when comparing different patients, can be evaluated in future studies.

Pigmented villonodular synovitis (PVNS) is a rare benign disorder of a joint which affects its synovium, tendon sheaths and bursas. While most cases of PVNS are adult patients aged between 20-50 years, few afflicted children have also been reported. Clinical signs of PVNS are insidious and non-specific. Pain, swelling and stiffness are the major symptoms. Magnetic Resonance Imaging (MRI) is the best radiological method for diagnosis of PVNS, as the initial X-ray is normal in early phase of disease in most cases. Therefore, diagnosis is often delayed or confused with mechanical disorders, haemophilic arthropathy, tuberculosis, juvenile idiopathic arthritis (JIA), and other disorders. Four paediatric PVNS cases are being reported in this case series with the aim to highlight that PVNS should be considered in the differential diagnoses of chronic monoarthritis. Two of our cases were initially misdiagnosed as JIA and the remaining two as Familial Mediterranean fever (FMF). They did not respond to conventional anti-inflammatory treatment and eventually only benefited from surgery. These four cases emphasize that the radiologist and clinician should collaborate carefully while managing any child with monoarthritis to ensure that the diagnosis of PVNS is not missed.

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Turkish language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach’s alpha, interscale correlations, test–retest reliability, and construct validity (convergent and discriminant validity). A total of 466 JIA patients (13.7% systemic, 40.6% oligoarticular, 22.5% RF negative poly-arthritis, and 23.2% other categories) and 93 healthy children were enrolled in four centres. The JAMAR components discriminated well-healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Turkish version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Purpose To collect retrospective data of patients with Juvenile Idiopathic Arthritis (JIA) and other rheumatic diseases who received live attenuated booster measles-mumps-rubella (MMR) or measles-mumps-rubella-varicella (MMR/V) during treatment with immunosuppressive therapy. Results Data from 13 pediatric rheumatology centers in 10 countries, including 234 patients, were collected. Mean age at diagnosis was 5 ± 2.7 years, 67% were girls. Among them, 211 (90.2%) had JIA and 110 (47%) were in remission on medication. Disease activity was low in 37%, high in 8%, and moderate in 8%. One hundred-twenty-four received MMR/V booster while on methotrexate (MTX); 3 reported local mild adverse events (AE). Among 62 on MTX + biologics and 9 patients who received a combination of 2 disease modifying antirheumatic drugs (DMARDs), 9 reported mild AE. Among 39 on biologics, 1 reported fever one day after booster vaccination. No vaccine-related infection of measles, rubella, mumps or varicella was reported, none of the patients developed disease flare, including those with high disease activity. Conclusions In this retrospective study, live-attenuated MMR/V booster vaccines were safe for children with rheumatic diseases, on immunosuppressive therapies. This strengthens the Paediatric Rheumatology European Society (PReS) recommendation that vaccination with live attenuated vaccines in patients on immunosuppressive therapies can be considered individually, weighing the benefit of vaccination against the risk of inducing infection through vaccination. These data provide the basis for a prospective data collection study, planned by the PReS vaccination study group.

OBJECTIVE: To develop a composite DAS for JDM and provide preliminary evidence of its validity. METHODS: The Juvenile DermatoMyositis Activity Index (JDMAI) is composed of four items: physician's global assessment of overall disease activity ; parent's/child's global assessment of child's wellbeing ; measurement of muscle strength ; and assessment of skin disease activity. The score of the JDMAI is the arithmetic sum of the scores of each individual component. Six versions of the JDMAI were tested, which differed in the tools used to assess the third and fourth items. Validation procedures were conducted using three large multinational patient samples including a total of 627 patients. RESULTS: The JDMAI was found to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha = 0.58- 0.89), fair responsiveness to clinically important change (standardized response mean = 0.82-3.12 among patients improved) and strong capacity to discriminate patients judged as being in the state of inactive disease or low, moderate or high disease activity by the physician (P < 0.001) or whose parents were satisfied or not satisfied with the course of their child's illness (P < 0.001). Overall, the six versions of the JDMAI showed similar metrological performances in validation analyses. CONCLUSION: The JDMAI was found to possess good measurement properties in a large population of patients with a wide range of disease activity, and is, therefore, suitable for use in both clinical and research settings. The final version of the JDMAI will be selected after its prospective validation

Background: Little is known about the long-term safety of canakinumab about tuberculosis (TB) risk in paediatric rheumatologic diseases. Objectives: To determine the incidence of latent tuberculosis infection and evaluate the follow-up protocol of the patients treated with canakinumab in a single pediatric rheumatology center from a TB-medium burden country. Methods: The hospital charts of patients treated with canakinumab between 2012 and 2019 were retrospectively reviewed. The patients were screened for TB using tuberculin skin test (TST) and/or QuantiFERON-TB Gold (QFT-G) test. They had no history of active TB prior to screening and no signs or symptoms of active TB. None of the patients had recent close contact with a person diagnosed as active TB. At initial evaluation, in patients who had never been given immunosuppressive therapy, TST ≥15 mm (if BCG vaccinated) and TST ≥10 mm (if BCG unvaccinated) were considered as positive. In the case of prior immunosuppressive drug use TST cut-off limit was accepted as 5 mm. Patients with either a positive TST or QFT-G result, in whom active TB ruled out, were to receive appropriate treatment for latent TB one month prior to the first dose of canakinumab and continue throughout 9 months. Chest radiographs were performed prior to therapy and every 6 months. In case TST was anergic, both chest X-ray and TST were repeated after 3 months. Patients were evaluated for signs and symptoms of active TB by an infectious disease specialist every 3 months while on canakinumab therapy according to the “guidelines of Ministry of Health for use of biologic agents”. The TST conversion was considered as a variation ≥5 mm between the two TSTs performed within an interval at least 3 months. If TB was suspected during the study, repeat TB testing (TST, QFT-G, and chest radiograph) was recommended. Results: 29 Familial Mediterranean fever, 6 systemic juvenile idiopathic arthritis, 1 TRAPS and 1 hyper Ig D syndrome patients were evaluated. The mean age at canakinumab onset was 12.4 ± 4.3 years (min: 2, max: 17 years). The average duration of canakinumab use was 2.3±1.4years (min: 1 max: 6.5 years). Among 37 patients, 5 patients had positive TST and one had positive QFT-G with normal chest X-ray prior to therapy. They were given isoniasid prophylaxis for latent TB. QFT-G was checked in 6 patients prior to canakinumab, 5 were negative. Seven patients had TST conversion during follow-up. Their chest X-rays were normal. None of them had active TB symptoms, such as; cough, fever, night sweats and weight loss. The mycobacterial cultures, mycobacterium tuberculosis PCR of induced sputum (via hypertonic saline nebulization) or morning gastric aspirates were negative in these patients. QFT-G was checked in 10 patients during follow-up and all were negative. None of the patients experienced active TB during follow-up. Conclusion: To the best of our knowledge, this is the first study investigating the frequency of latent tuberculosis infection among patients treated with canakinumab. The results of this study suggest that although frequency of latent TB infection in children treated with canakinumab may not be low in a TB-medium burden country, canakinumab seems to be a safe treatment option in terms of active tuberculosis risk. Close follow-up of children on canakinumab treatment with respect to TB by the pediatric infectious disease department is important to prevent possible complications. Reference: [1] T.C Saglik Bakanligi Turkiye Halk Sagligi Kurumu AntiTNF Kullanan Hastalarda Tuberkuloz Rehberi. Available at: http://www.mhsm.gov.tr/images/diger_haberler/anti_tnf_kullanan_hasta_tb_rehber.pdf. Disclosure of Interests: Balahan Makay Speakers bureau: Enzyvant, Novartis, Roche, Abbvie, ozge altug gucenmez Speakers bureau: Novartis, Abbvie, Ilknur Caglar: None declared, Suleyman Nuri Bayram: None declared, Nesrin Gulez: None declared, Ilker Devrim: None declared

Background Common practice is to withhold vaccination with live-attenuated vaccines in patients with rheumatic diseases on high-dose DMARDs, glucocorticosteroids or biological agents, due to limited safety data, and the (theoretical) risk of introducing an infectious disease to the patient. Evidence for this approach is low. We collected data from pediatric rheumatologists who vaccinate these patients, to obtain additional safety data, which might update and revise this approach. Objectives To collect retrospective data in patients with JIA and other diseases who received live booster MMR or MMRV while on DMARDs, glucocorticosteroids or biological agents. Methods Data from 13 pediatric rheumatology centers in 10 countries were collected. Results 234 patients were reported; mean age 5± 2.7, 70% girls. 206 had JIA;. 46% oligoarticular, 36% polyarticular, 8% systemic, 5% SPA types, 5% JIA and uveitis. 48% of JIA patients were in remission on medication. Disease activity was low in 38%, high in 2%, moderate in 7%; 11 patients had juvenile dermatomyositis, 3 systemic and 2 localized scleroderma, 4 isolated idiopathic uveitis, 1 CINCA syndrome, 1 MKD, and 1 FMF. 110 patients had MMR/V booster while on MTX; 3 reported mild side-effects of local skin reaction and pain, none had disease flare. 76 had booster while on MTX+ anti-TNF; 7 reported mild and transient adverse events of local skin reaction, fever and URTI. 39 had booster while on anti-TNF alone; 1 reported fever. 3 had booster while on tocilizumab, 7 on anakinra, and 5 on canakinumab. There was no relation between disease activity, type or duration, sex, age and outcome of vaccinations. No vaccine infection related to measles, rubella, mumps and varicella were reported. Conclusion This large, retrospective data collection demonstrates that live-attenuated booster vaccine is probably safe in children with rheumatic diseases, on immunosuppressive therapies. This strengthens the new PRES recommendation: “Vaccination of live-attenuated vaccines in patients on high-dose DMARD, high-dose glucocorticosteroids or biological agents can be considered on a case-by-case basis, weighing the risk of infections against the hypothetical risk of inducing infection through vaccination.” These data provide the basis for a large, prospective data collection study that is planned by the PReS vaccination study group. It will increase the current level of evidence for the safety of vaccinations in our pediatric rheumatology population. Disclosure of Interests Yosef Uziel: None declared, Veronica Moshe Bergonzo: None declared, Beata Onozo: None declared, Andrea Kulcsar: None declared, Diana Trobert-Sipos : None declared, Jonathan Akikusa: None declared, Gecilmara Salviato Pileggi : None declared, Despoina Maritsi: None declared, Ozgur KASAPCOPUR: None declared, Roubini Smerla: None declared, Donato Rigante: None declared, Erato Atasali: None declared, Mariana Rodrigues: None declared, Balahan Makay Speakers bureau: Enzyvant, Novartis, Roche, Abbvie, Nico Wulffraat: None declared, Natasa Toplak: None declared

Background Familial Mediterranean fever (FMF) is the most common monogenic periodic fever syndrome. The MEFV gene mutation encoding the pyrin protein results in an uncontrolled increase in interleukin-1. Today, more than 333 MEFV mutations have been identified; however, exon 10 mutations are still seem to be best correlated with clinical findings. Objectives In this study, we aim to investigate the role of exon 10 mutations vs non-exon 10 mutations on clinical features and response to treatment in patients with FMF. Methods Data charts of children (n=935) with FMF from Dokuz Eylul University childrens’ hospital and Dr.B.Uz childrens’ hospital were reviewed. Patients were divided into two groups with regard to having exon 10 mutation or non-exon 10 mutations. Genotype-phenotype features and response to treatment were compared. Results There were exon 10 mutations in 631 (67.5%) patients and non-exon 10 mutations in 304 (32.5) patients. The follow-up period was 50 (26-83.2) months. The age of symptoms onset was significantly lower in group with exon 10 positive than compared group with non-exon 10 mutation. There was no difference between the age of diagnosis and colchicine onset and the diagnosis delay time. The symptoms of fever, chest pain, and arthritis were significantly higher in the exon 10 mutation group than compared other group. Biological agent need was statiscally higher in exon 10 mutation group (4.8%) than group with non-exon 10 mutation (1.3%) (Table 1). Conclusion In our study, it was observed that cases with exon 10 mutation have early symptoms of disease. Fever, chest pain and joint findings were more prominent in cases with exon 10 mutation than cases with non-exon 10 mutation. Additionally, colchicine resistance should be kept in mind in cases with exon 10 mutation. References [1] Wang DQH, Bonfrate L, de Bari O, Wang TY, Portincasa P (2014) Familial Mediterranean fever: from pathogenesis to treatment. J Genet Syndr Gene Ther5:248. [2] Ozen S, Demirkaya E, Amaryan G, et all. Results from a multicentre international registry of familial Mediterranean fever: impact of environment on the expression of a monogenic disease in children. Ann Rheum Dis. 2014Apr;73(4):662-7 Disclosure of Interests Hatice Adiguzel Dundar: None declared, ozge altug gucenmez Speakers bureau: Novartis, Abbvie, Ceyhun Acari: None declared, Serkan Turkucar: None declared, Balahan Makay Speakers bureau: Enzyvant, Novartis, Roche, Abbvie, Erbil Unsal Grant/research support from: Novartis, AbbVie, Roche, Kocak Pharma, Speakers bureau: Novartis, AbbVie, Roche, Kocak Pharma

Background Assessment of muscle strength is a fundamental component of the clinical evaluation of children with juvenile dermatomyositis (JDM). Regular measurement of muscle strength in daily care requires the availability of simple and quick muscle assessment tools. Objectives To investigate whether reduced versions of the MMT8 and CMAS are equally reliable as the original tools. Methods The following 4 reduced instruments were devised: 1) MMT4 (score 0-40), including 4 items of MMT8 (neck flexors, deltoid middle, gluteus maximus, and gluteus medius); 2) MMT6 (score 0-60), composed of the same items of MMT4 plus biceps brachii and quadriceps; 3) head lift time of CMAS (0-120 seconds or 0-5 points); 4) sum of CMAS head lift time in points and the 6 sit-ups maneuvers of CMAS (score 0-11). Validation was conducted according to OMERACT filter on 213 patients followed in standard clinical care at 13 international pediatric rheumatology centers and evaluated at baseline and after a median of 5.9 months. Results All reduced instruments revealed strong correlations (r > 0.7) with muscle activity VAS and total DAS, moderate correlations (r = 0.4-0.7) with physician’s global VAS, muscle DAS, skin activity VAS, pain VAS, parent’s overall wellbeing VAS, and CK. Correlations with skin DAS and fatigue VAS were low (r Conclusion We found that reduced versions of the MMT8 and CMAS have good metrologic properties and perform similarly to the original tools in a population of patients followed in standard clinical care. Our results suggest that these simplified and shortened instruments could serve as surrogate for the complete measures in routine practice, particularly in a busy clinical setting. References [1] Measures of adult and juvenile dermatomyositis, polimyositis, and inclusion body myositis. Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, et al. Arthritis Care Res (Hoboken)2011;63:S118-57. [2] Clinical assessment in juvenile dermatomyositis. Ravelli A, Ruperto N, Trail L, Felici E, Sala E, et al. Autoimmunity2006;39:197-203. Disclosure of Interests Silvia Rosina: None declared, Giulia Camilla Varnier: None declared, Angela Pistorio: None declared, Alessandro Consolaro Grant/research support from: AbbVie, Pfizer, Clarissa Pilkington: None declared, Susan Maillard: None declared, Adele Civino: None declared, Elena Tsitsami: None declared, Jaime de Inocencio: None declared, Marija Jelusic: None declared, Jelena Vojinovic: None declared, Graciela Espada: None declared, Balahan Makay Speakers bureau: Enzyvant, Novartis, Roche, Abbvie, Maria Katsikas: None declared, Polixeni Pratsidou-Gertsi: None declared, Dragana Lazarevic: None declared, Anand Prahalad Rao: None declared, Nicolino Ruperto Grant/research support from: The Gaslini Hospital, where NR works as full-time public employee, has received contributions (> 10.000 USD each) from the following industries in the last 3 years: BMS, Eli-Lilly, GlaxoSmithKline, F Hoffmann-La Roche, Janssen, Novartis, Pfizer, Sobi. This funding has been reinvested for the research activities of the hospital in a fully independent manner, without any commitment with third parties., Consultant for: Received honoraria for consultancies or speaker bureaus (

Familial Mediterranean fever (FMF) is an inherited autoinflammatory disorder that can result in attacks with accompanying recurrent episodes of fever, serositis, and skin rash. MiRNAs are demonstrated to be associated with a number of other diseases; however, no comprehensive study has revealed its association with FMF disease. The aim is to investigate the role of microRNAs in FMF. We included 51 patients with genetically diagnosed FMF who had clinical symptoms and 49 healthy volunteers. Fifteen miRNAs that were found to be associated with autoinflammatory diseases and have a part in immune response were evaluated. The expression levels of 11 miRNAs (miR-125a, miR-132, miR-146a, miR-155, miR-15a, miR-16, miR-181a, miR-21, miR-223, miR-26a, and miR-34a) in the patient group were significantly low, compared with the control group (p

Background:Behçet disease (BD) is a systemic inflammatory disease which is rare in children. It is also known a higher prevalence along the ancient Silk Road and the eastern Mediterranean, especially in Turkey. Despite the presence of diagnostic criteria, the diagnosis of pediatric BD is still difficult due to atypical findings and the heterogeneity of the disease. While descriptive cohort studies in pediatric BD exist, direct comparison studies of clinical manifestations and disease activity between patients from different countries are scarce.Objectives:We aimed to compare the main clinical features and activity of pediatric BD patients from Turkey versus United States (US).Methods:The BD was diagnosed before 18 years of age and based on expert opinion. Disease activity was assessed with Physician Global Assessment (PhGA), Parents/Patient Global Assessment (PWPGA) and Behçet’s Syndrome Activity Scale (BSAS) were administered to patients from both countries.Results:A total of 161 patients were included (61 from Turkey; 100 from US). Males were more prevalent among patients from Turkey than among patients from US (63.9% vs. 32%, respectively, p < 0.001). Disease duration at the diagnosis was significantly longer in US p=0.002). Oral aphthosis was the most common symptom in both groups (96.7% for Turkey and 73% for US), however a significant difference was found (pConclusion:Disease activity and clinical features seem to be different between the two countries which may be linked to the environmental factors, referral patterns and immune system responses in the expression of this disease. The diagnosis of BD in the US was based on both fulfilling the ISBD criteria and in cases when criteria were not met, based on treating physician assessment, which can explain some of the differences in disease presentation. In addition, there were differences in regard to clinical practice and treatment patterns between two countries. Increasing knowledge about heterogeneity of BD will improve the ability of diagnosis, development of new diagnostic criteria, and management of BD.References:[1]Butbul Aviel Y et al, Semin Arthritis Rheum. 2020Disclosure of Interests:None declared

Acid Ceramidase Deficiency (Farber disease, FD) is an ultra-rare Lysosomal Storage Disorder that is poorly understood and often misdiagnosed as Juvenile Idiopathic Arthritis (JIA). Hallmarks of FD are accumulation of ceramides, widespread macrophage infiltration, splenomegaly, and lymphocytosis. The cytokines involved in this abnormal hematopoietic state are unknown. There are dozens of ceramide species and derivatives, but the specific ones that accumulate in FD have not been investigated. We used a multiplex assay to analyze cytokines and mass spectrometry to analyze ceramides in plasma from patients and mice with FD, controls, Farber patients treated by hematopoietic stem cell transplantation (HSCT), JIA patients, and patients with Gaucher disease. KC, MIP-1 alpha, and MCP-1 were sequentially upregulated in plasma from FD mice. MCP-1, IL-10, IL-6, IL-12, and VEGF levels were elevated in plasma from Farber patients but not in control or JIA patients. C16-Ceramide (C16-Cer) and dhC16-Cer were upregulated in plasma from FD mice. a-OH-C18-Cer, dhC12-Cer, dhC24:1-Cer, and C22:1-Cer-1P accumulated in plasma from patients with FD. Most cytokines and only a-OH-C18-Cer returned to baseline levels in HSCT-treated Farber patients. Sphingosines were not altered. Chitotriosidase activity was also relatively low. A unique cytokine and ceramide profile was seen in the plasma of Farber patients that was not observed in plasma from HSCT-treated Farber patients, JIA patients, or Gaucher patients. The cytokine profile can potentially be used to prevent misdiagnosis of Farber as JIA and to monitor the response to treatment. Further understanding of why these signaling molecules and lipids are elevated can lead to better understanding of the etiology and pathophysiology of FD and inform development of future treatments. (C) 2016 Elsevier B.V. All rights reserved.

Exercise capacity has been reported to be lower in children with Juvenile Idiopathic Arthritis (JIA). Therefore, the aim was to investigate the effects of an 8-week water-based exercise program on exercise capacity in children with JIA. Forty-two children with JIA were divided into two groups as: exercise group [n = 21, water-running, moderate-intensity exercise (60–70%), two times/week], and control group (n = 21, no additional treatment other than the prescribed medication). All children were assessed at baseline and post-intervention in terms of physical and disease-related characteristics, pain at rest and in activity (visual analog scale), range of motion (Escola Paulista de Medicina Range of Motion Scale), aerobic exercise capacity (cycle ergometer), and anaerobic exercise capacity (Wingate Test). Anaerobic exercise capacity was found to be improved in the exercise group [baseline: 5.54 W/kg (IQR 25/75: 4.07/6.88 W/kg) vs. post-intervention: 6.0 W/kg (IQR 25/75: 4.8/7.4 W/kg), p = 0.002], while no improvements were observed in the control group [baseline: 5.29 W/kg (IQR 25/75: 4.75/5.85 W/kg) vs. post-intervention: 5.5 watts/kg (IQR 25/75: 5.0/6.1 W/kg), p = 0.076]. The amount of the changes related to anaerobic exercise capacity were higher in the exercise group [exercise group: 0.6 W/kg (IQR 25/75: 0.3/1.3 W/kg) vs. control group: 0.2 W/kg (IQR 25/75: − 0.1/0.5 W/kg), p = 0.024]. No changes were detected related to aerobic exercise capacity in any of the groups (p > 0.05). An 8-week water-running program might be beneficial to improve anaerobic exercise capacity, but it is not enough to improve the aerobic exercise capacity in children with JIA.

Altug-Gucenmez O, Makay B, Kacar A, Unsal E. Evaluation of restless legs syndrome and growing pains in children with familial Mediterranean fever. Turk J Pediatr 2018; 60: 159-164. Growing pains (GP) and restless leg syndrome (RLS) are one of the frequently seen pain syndromes of childhood. These two pain syndromes -GP and RLS- may be confused with exertional leg pain (ELP) of familial Mediterranean fever (FMF). The aim of this study was to evaluate the frequency of fulfilling the criterion for GP and RLS among children with FMF. Sixty FMF patients and 70 healthy controls were enrolled. Clinical and demographic data of patients were recorded. A questionnaire including the symptoms of GP and RLS were applied to all participants and their parents. Twenty-seven patients (45%) had ELP. Ten FMF patients and 10 healthy children fulfilled GP criteria. There was not a significant difference between patients and controls regarding GP. Three FMF patients and 7 healthy controls fulfilled RLS criteria. There was not a significant difference between two groups regarding RLS. Two out of 27 FMF patients with ELP had RLS while one out of 33 FMF patients without ELP had RLS. Eight out of 27 FMF patients with ELP fulfilled GP criteria while two out of 33 FMF patients without ELP fulfilled GP criteria (p=0.01). There was a significant inverse correlation with FMF severity score and GP (p=0.003 and r=-0.376). There was not a significant association with FMF severity score and RLS. This study suggested that GP and RLS are not more common in pediatric FMF patients than their healthy peers.

BackgroundPrevious studies investigating the known risk factors of atherosclerosis in phenylketonuria patients have shown conflicting results. The primary aim of our study was to investigate the serum atherogenic markers in adolescent classical phenylketonuria patients and compare these parameters with healthy peers. The secondary aim was to compare these atherogenic markers in well-controlled and poorly controlled patients.MethodsA total of 59 patients (median age: 12.6 years, range: 11–17 years) and 44 healthy controls (median age: 12.0 years, range: 11–15 years) were enrolled in our study. Phenylketonuria patients were divided into two groups: well-controlled (serum phenylalanine levels below 360 µmol/L; 24 patients) and poorly controlled patients (serum phenylalanine levels higher than 360 µmol/L).ResultsThe mean high-density lipoprotein cholesterol levels of well-controlled patients (1.0±0.2 mmol/L) were significantly lower compared with poorly controlled patients and controls (1.1±0.2 mmol/L, p=0.011 and 1.4±0.2 mmol/L, pConclusionLower high-density lipoprotein cholesterol and higher homocysteine and mean platelet volume levels were detected in phenylketonuria patients. In particular, these changes were more prominent in well-controlled patients. We conclude that phenylketonuria patients might be at risk for atherosclerosis, and therefore screening for atherosclerotic risk factors should be included in the phenylketonuria therapy and follow-up in addition to other parameters.

Objective To develop and test a hybrid measure of muscle strength for juvenile dermatomyositis (JDM), which is based on the combination of the Manual Muscle Testing 8 (MMT8) and the Childhood Myositis Assessment Scale (CMAS), but is more comprehensive than the former and more feasible than the latter. Methods The hybrid MMT/CMAS (hMC) is composed of all 8 items of the MMT8 and 3 items of the CMAS: 1) time of head lift; 2) assessment of abdominal muscles; 3) floor rise. The score ranges from 0 to 100, with 100 indicating normal muscle strength. Validation procedures were conducted using three large multinational patient samples including a total of 810 JDM patients. Results The hMC revealed face and content validity, good construct validity, excellent test-retest reliability (intraclass correlation coefficient = 0.99) and internal consistency (Chronbach's alpha = 0.94), strong responsiveness to clinical change over time (standardized response mean = 0.8 among patients judged as improved by the caring physician), and satisfactory capacity to discriminate patients judged as being in the states of inactive disease or low, moderate, or high disease activity by the physician (p < 0.001) or patients whose parents were satisfied or not satisfied with illness course (p < 0.001). Conclusion The hMC was found to possess good measurement properties in a large population of patients with a wide range of disease activity and severity. The new tool, which is primarily intended for use in routine clinical care, should be further tested in other populations of patients evaluated prospectively. This article is protected by copyright. All rights reserved.

Aims This study aimed to investigate sleep patterns, sleep disturbances and possible factors that are associated with sleep disturbances among children with familial Mediterranean fever (FMF). Patients and methods Fifty-one patients with FMF and 84 age- and sex-matched healthy controls were enrolled in the study. The patients who had an attack during the last 2 weeks were not included. Demographic data, FMF symptoms, disease duration, dose of colchicine, disease severity score, number of attacks in the last year, MEFV mutation and serum C-reactive protein (CRP) levels were recorded for each patient. A Children's Sleep Habits Questionnaire was performed. Results The total sleep scores of the patients with FMF were significantly higher than the control group. Total sleep durations were similar between FMF patients and controls. Children with FMF had significantly higher scores regarding sleep-onset delay, sleep anxiety, night wakings and sleep-disordered breathing when compared to healthy controls. There was a significant positive correlation between number of attacks in the last year and sleep onset delay, night wakings and sleep disordered-breathing. Disease severity score and CRP levels were not associated with any of the subscale scores. The patients with exertional leg pain had significantly higher total sleep scores than the ones without. Furthermore, patients with exertional leg pain had significantly higher subscale scores regarding sleep onset delay, parasomnias and sleep-disordered breathing. Conclusion This study showed for the first time that children with FMF had more sleep disturbances than their healthy peers. Higher numbers of attacks and exertional leg pain were associated with poor sleep quality. In conclusion, this study underlines the need to assess and manage sleep problems in children with FMF.

AIM Familial Mediterranean fever (FMF) is the most common auto-inflammatory disease with recurrent fever and serositis episodes. In recent years, some cases with FMF were reported with gastrointestinal involvement without amyloidosis, vasculitis and inflammatory bowel disease (IBD). It is not yet known whether gastrointestinal involvement is a part of the disease or not. The aim of this study is to investigate the frequency of intestinal inflammation by using a noninvasive method, fecal calprotectin measurement, in pediatric FMF patients. METHOD Sixty-five FMF patients, 30 healthy controls and 11 patients with acute ulcerative colitis were included in the study. A standard survey inquiring gastrointestinal and other clinical symptoms was completed. The medications, MEFV mutations, whole blood count and C-reactive protein levels were recorded. Fecal calprotectin was studied with the enzyme-linked immunosorbent assay method from the feces samples of the all subjects. RESULTS None of the FMF patients had clinical signs of IBD. Fecal calprotectin levels of the FMF patients were found to be significantly higher than the healthy controls (174.8 ± 150.8 vs 52.9 ± 36.5, p

Although familial Mediterranean fever (FMF) is inherited autosomal recessively, some heterozygotes may express disease phenotype and require therapy. To date, there is no study in the literature about how to follow-up Mediterranean fever (MEFV) heterozygotes who do not fulfil FMF criteria in the paediatric age group. This study aims to share a single-centre experience of the long-term clinical and laboratory follow-up of paediatric MEFV carriers. We reviewed the charts of 69 children who were heterozygous for MEFV variants. All children were followed-up with their routine analysis and serum amyloid A levels every 6 months. Thirty-nine children had pathogenic mutations and 30 children had variants of unknown significance. The mean follow-up was 3.2 ± 1.6 years (min 2 years, max 6 years). The children with pathogenic mutations had significantly higher mean SAA levels than the children with variants of unknown significance (p = 0.018); however, the mean CRP and ESR were similar. Besides, the children with pathogenic mutations complained of fever episodes significantly more than the children with variants of unknown significance (p = 0.04). None of the children had persistent proteinuria in the follow-up. We started colchicine in only two patients who were M694V heterozygous. Both patients had family history for FMF and fulfilled the disease criteria after 2 years of follow-up. Neither of these patients had persistently elevated acute phase reactants in their routine follow-up. This study suggested that routine clinical follow-up is useful; however, routine periodic laboratory workup is not necessary among MEFV carriers.

Background Juvenile idiopathic arthritis (JIA) is a chronic disease that occurs before the age of 16 years. It was shown that anaerobic exercise capacity, which is important for most daily activities in children such as jumping, hoping and climbing was diminished in JIA. Previous studies showed that anaerobic exercise capacity was related to well-being level, functional status, and aerobic exercise capacity in JIA. However, no data is available about the relationship between lower extremity muscle power and anaerobic exercise capacity in children with JIA. Objectives To determine the possible relationships between lower extremity muscle strength and anaerobic capacity. Methods Forty-six children with JIA (14 F, 32 M), with a mean age of 13.74±2.29 years (min-max: 9–17 years) were included in the study. Isometric lower extremity muscle strength was assessed with a hand-held dynamometer at the end points of knee flexion, knee extension, hip flexion and hip extension movements, which are generated from gross lower extremity muscles and important for anaerobic power. All the muscle testing was performed on the right leg. Anaerobic exercise capacity was measured performing a 30-second Wingate test. Both absolute and per kilogram values for peak power and average power were noted. The relationships between the parameters were determined with Pearson9s correlation coefficient. (Relationship levels were interpreted as 0.05–0.30: low correlation; 0.30–0.40: low-moderate correlation; 0.40–0.60: moderate correlation; 0.60–0.75: good correlation; 0.75–1.00: good-excellent correlation. Results All children completed the assessments without any adverse effects. Demographics, average isometric lower extremity muscle strengths and parameters related to the anaerobic exercise capacity testing were shown at Table 1. Moderate to good correlations were determined between isometric muscle strength and anaerobic exercise capacity parameters p Conclusions The results of this study suggested that lower extremity muscle strength might influence the anaerobic exercise capacity. Exercise regimes including lower extremity strengthening might help improving anaerobic exercise capacity in children with JIA. Disclosure of Interest None declared

Background The management of juvenile idiopathic arthritis (JIA) includes various methods including such as medication, hospitalization, rehabilitation. Objectives To determine how much juvenile idiopathic arthritis cost; the components of this cost; how new treatments, i.e. biologics, improve the disease course and hospital expenditures. Methods This study was conducted in Dokuz Eylul University, Pediatric Rheumatology Unit between March 2015-March 2016. One-hundred six JIA patients who had a follow-up period of at least 1 year according to International Edmonton 2001 criteria were included. This retrospective cost study evaluated the data of these patients and calculated the direct cost for the follow-up period. Clinical data was collected from patient files that were in department9s archive and cost data was gathered from Probel Hospital Information Management system. Patient data form covering sociodemographic and clinical information, patient drug form and annual medical cost form was filled out for each patient. Results 58.5% (n=62) of patients was female and 41.5% (n=44) was male. The mean age was 12.0±4.3 years. 34.0% (n=36) of patients was oligoarticular type, 28.3% (n=30) was poliarticular type, 22.6% (n=24) was enthesitis related arthritis (ERA), 8,5% (n=9) was psoriatic type and 6.6% (n=7) was systemic type. The cost of medication counted for 88.3% (453244.94 TL) of total direct annual cost. Total direct medical cost was highest for ERA (n=7742.55±9891 TL). While the annual cost was calculated as 10451 TL per person for biologic using patients, for the patients using non-biologic treatments it was determined as 1472 TL per person. 1 TL=0.32 € 1 TL =0.35 $ Conclusions Medication is responsible for most of the total direct medical cost in patients with JIA. Our results showed concordance with previous studies on the subject. This situation could be attributed to biologic agents that are being used in treatment in recent years. More prospective studies on the effectiveness of cost of treatment, with greater amount of patient and more homogenous subgroups are needed. Disclosure of Interest None declared

Background Anaerobic exercise capacity was reported to be lower in children with juvenile idiopathic arthritis (JIA) than healthy children. However, to our knowledge, there is no study focusing on improving anaerobic exercise capacity in JIA. Objectives To investigate the effect of an 8-week water exercise program, which was performed at the weekends, on anaerobic exercise capacity in children with JIA. Methods Forty-two children with JIA were divided into two groups as exercise and control. Prior to the study, anaerobic exercise capacity was measured performing a 30-second Wingate test. Deep water running was employed as the progressive water exercise program for the exercise group. Control group did not receive any additional treatment other than their usual care. Weekends were chosen for the exercise sessions considering the educational program of the children. Exercise intensity was set as moderate. Exercise intensity was determined with a wearable heart rate tracking system during the exercises. All children were reassessed regarding to anaerobic exercise capacity two months after the first assessment. Results All children completed the study without any adverse effects. Twenty-one children were in the exercise group, others were assessed as controls. No significant differences were determined between groups prior the study regarding to age, disease duration, height, weight, body-mass index, and anaerobic exercise capacity related parameters (p Conclusions The present study is the first study focusing on improving anaerobic capacity in children with JIA. According to our results, an 8-week water exercise program which is performed at the weekends might be beneficial to improve anaerobic exercise capacity in children with JIA. Disclosure of Interest None declared

15th Annual Research Meeting of the WORLDSymposium(TM) -- FEB 04-07, 2019 -- Orlando, FL WOS: 000457351700338 … WORLDSymposium

Objectives To determine the frequency of vitamin D deficiency in children with familial Mediterranean fever (FMF) and to investigate the factors associated with low vitamin D status. Design and methods Forty-four patients with FMF and 39 age- and sex-matched healthy controls were enrolled in this study. Demographic data, FMF symptoms, disease duration, time to delay for diagnosis, duration of follow-up, disease severity score, MEFV gene mutation, cumulative colchicine dose, compliance to treatment and serum C-reactive protein levels were recorded for each patient. Serum 25-hydroxyvitamin D levels were measured by an original commercial kit based on chemiluminescent microparticle immunoassay (CMIA). Results The serum 25-hydroxyvitamin D levels were significantly lower in FMF patients than the healthy controls (12.9 ± 3.6 and 16.3 ± 5.5 ng/mL, respectively, P = 0.001). Vitamin D levels were similar in patients homozygous for M694V and other genotypes (11.8 ± 3.7 and 13.2 ± 3.6 ng/mL, respectively, P = 0.21). Stepwise multiple linear regression analysis confirmed that the cumulative colchicine dose was the strongest independent variable correlating with vitamin D levels (r2 = 0.194, P = 0.001). Conclusion Our results suggest that serum 25-hydroxyvitamin D levels are decreased in children with FMF. Cumulative colchicine dose appears to negatively affect vitamin D levels. The role of colchicine on vitamin D metabolism needs to be elicited.

Aims This study aims to assess the correlation of mean platelet volume (MPV) and common carotid artery (CCA) thickness in a population of obese adolescents. Methods Sixty-eight patients and 23 controls were enrolled. Anthropometric measurements, triglyceride, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase, hemoglobin, white blood cell count, platelet count, MPV and insulin resistance by homeostasis model of assessment of insulin resistance were investigated. Furthermore, CCA thickness was measured by high-resolution ultrasound. Results MPV and the left CCA thickness were significantly higher in obese adolescents than the healthy controls. The association between MPV and left CCA was checked by linear regression analysis. MPV explained 19% of the variation in left CCA (P < 0.001). At multiple regression analysis, MPV maintained a positive association with the left CCA thickness (P = 0.002) independently of fatty liver grade, relative weight, total cholesterol and homeostasis model of assessment of insulin resistance. Conclusion MPV is significantly correlated with CCA thickness in obese adolescents.

Objectives This study aims to evaluate the publication rates and features of the abstracts related to pediatric rheumatology presented in European League against Rheumatism 2009 congress. Patients and methods A systematic search was performed to find full-text publications of abstracts related to pediatric rheumatology in European League against Rheumatism 2009 congress. Full-text publication rate, the elapsed time between presentation and full-text publication, type of the disease in the studies, distribution of abstracts and full-texts according to countries, journals, and presentation types were investigated. Results Totally 220 abstracts were detected related to pediatric rheumatology. Twenty-two of them had only the title, authors' names, and institution of origin, but no abstract. Therefore, a total number of 198 abstracts were evaluated. Eighty-six (43.4%) abstracts were found to be accepted as full-text articles. The elapsed time between presentation and full text publication was median 19 months (range 0 to 64 months). While 12 orally presented abstracts (34%) became full-text articles, this rate was 45% (74 abstracts) for poster presentations. There was no significant difference in the elapsed time to reach full-text publication and impact factors between presentation types (p=0.832 and p=0.053, respectively). Conclusion The full-text publication rates were within similar ranges when compared to other reports in rheumatology field. It seems that even though European League against Rheumatism is a general rheumatology congress, it takes an important place in pediatric rheumatology field as well.

The name of the last author of this article was incorrectly presented as "Cogulu Ozgur" this should have been "Ozgur Cogulu".

Familial Mediterranean fever (FMF) is the most common inherited periodic fever syndrome characterized by recurrent episodes of serositis. Recently, a few studies have suggested that FMF is related to increased risk of atherosclerosis. Mean platelet volume (MPV) is a marker of platelet activation. Larger platelets are associated with increased atherosclerosis risk. The aim of the study is to evaluate levels of MPV in pediatric FMF patients during and between attacks. The study consisted of 48 patients during an attack (group 1), 63 patients in attack-free period (at least 2 weeks after an attack, group 2), and 49 healthy controls (group 3). Erythrocyte sedimentation rate, C-reactive protein, white blood cell count, platelet count (PLT), and MPV levels were retrospectively recorded from the computerized patient database. Mean platelet volume was significantly lower in FMF patients during attack than in attack-free period (p = 0.00); however, there was no difference among attack-free patients and healthy controls (p = 0.38). The mean platelet counts of FMF patients during attack were higher than the healthy controls (p = 0.02). There was an inverse correlation between MPV and mean PLT in the attack-free period (r = -446, p = 0.01). This study suggests that an early atherosclerosis marker, MPV, is not elevated in pediatric FMF patients on colchicine treatment.

Objective.Patients with enthesitis-related arthritis (ERA) were less likely to achieve and sustain inactive disease than children with other subtypes of juvenile idiopathic arthritis. The aim of this study was to evaluate the effect of increased body mass index (BMI) on clinical features of the disease and to investigate whether being overweight or obese limits the possibility of achieving clinically inactive disease in patients with ERA.Methods.The hospital charts of 72 patients with ERA were reviewed. Demographic and clinical findings were recorded. Patients were divided into 2 groups according to whether they had “healthy weight” (BMI < 85th percentile) or “increased weight” (BMI ≥ 85th percentile) at baseline. The primary outcome of this study was to achieve inactive disease at 1 year after the initiation of therapy. The inactive disease criterion of Wallace,et alwas used to define inactive disease status.Results.Twenty patients had increased BMI. The frequency of tarsitis and ankle involvement was higher in patients with increased weight. Thirty-seven patients were inactive at the end of 1 year. In univariate analyses, male sex, increased BMI, ankle involvement, and tarsitis were found to be associated with failure to achieve inactive disease. Multivariate backward stepwise regression analyses revealed that failure to achieve clinically inactive disease was associated with increased BMI and ankle involvement.Conclusion.Being overweight or obese was associated with failure to achieve inactive disease in patients with ERA. Because body weight is a modifiable factor, individualized interventions may have clinical implications for better therapeutic outcome.

We read with interest the study by Ozer et al.1 evaluating the association between colchicine resistance and vitamin D levels in familial Mediterranean fever (FMF). Patients with ≥ 3 attacks withi...

Methods Recommendations were established via consensus by a panel of experts in pediatric rheumatology, based on analysis of available scientific evidence obtained from the clinical experience of panelists. The Delphi method solicits the opinion of an expert panel through a carefully designed questionnaire which in this case included questions on: epidemiology, diagnosis, treatment of symptoms, drug choice, adverse events, follow-up visits, hospital and emergency service admissions. The responses were analyzed and discussed in a face to face meeting followed by consensus building steps.