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Özge Akgün,1 Christian Oudshoorn,1 Francesco US Mattace-Raso,1 Angelique Egberts1,2 1Department of Internal Medicine, Section of Geriatric Medicine, Erasmus MC University Medical Center, Rotterdam, the Netherlands; 2Department of Hospital Pharmacy, Franciscus Gasthuis & Vlietland, Rotterdam & Schiedam, the NetherlandsCorrespondence: Angelique Egberts, Section of Geriatric Medicine, Department of Internal Medicine, Erasmus MC University Medical Center, Room Rg-527, PO Box 2040, Rotterdam, CA, 3000, the Netherlands, Tel +31 10 70 35979, Fax +31 10 70 34768, Email a.egberts@erasmusmc.nlPurpose: In-hospital falls, especially among older patients, are a major and underestimated problem. Several studies have suggested a possible association between anticholinergic drug use and falls, but the results are inconclusive and studies focusing on in-hospital falls are scarce. The aim of the present study was to investigate whether anticholinergic drug exposure on admission is associated with in-hospital falls.Patients and Methods: This retrospective chart review study was conducted in the Erasmus MC University Medical Center, Rotterdam, the Netherlands. Patients aged 65 years and older, who were acutely admitted to the geriatric ward between 2012 and 2015, were included. Anticholinergic drug exposure was determined with the Anticholinergic Risk Scale (ARS), the Anticholinergic Cognitive Burden scale (ACB) and the list of Chew. Logistic regression was used to investigate the possible association between anticholinergic drug exposure and in-hospital falls. Analyses were adjusted for age, sex, fall history, fall as reason for admission, number of drugs on admission, use of a mobility aid and delirium.Results: A total of 905 patients were included, of which 94 patients experienced one or more in-hospital falls. Each additional anticholinergic drug in use, according to the ARS, was associated with an increased odd of experiencing a fall (OR = 1.49, 95% CI: 1.06– 2.10). Other measures, ie anticholinergic drug use (yes/no) and different categories of anticholinergic drug burden, measured with the ARS, ACB and list of Chew, were all not associated with in-hospital falls.Conclusion: Anticholinergic drug exposure on admission is possibly not a main risk factor for in-hospital falls among older patients.Keywords: accidental falls, cholinergic antagonists, inpatients

IntroductionThe employment of laparoscopic surgical techniques has reignited the debate on managing Meckel's Diverticulum (MD) due to its low complication rates. Nevertheless, concerns have been raised regarding completely removing any potential heterotopic mucosa. Our study aimed to compare surgical approaches in MD and assess the effectiveness of simple diverticulectomy.MethodsBetween 2003 and 2022, 139 patients with MD were retrospectively analysed. The study examined the morphometric measurements of the diverticulum and the location of the heterotopic mucosa in the diverticulum regarding growth and symptoms.ResultsSimple diverticulectomy achieved the lowest postoperative complication rate among excision techniques (p = 0.03). MD's length, diameter, and distance to the ileocecal valve increase linearly with growth in the first three years of life (p = 0.00, p = 0.01, p = 0.00) but not in subsequent years (p = 0.81, p = 0.43, p = 0.21). As the length of the MD increases, the heterotopic mucosa (HM) is displaced distally (p = 0.01). Patients in whom HM reaches the base of the diverticulum always present with bleeding (p = 0.02).DiscussionSimple diverticulectomy is a safe technique for Meckel's diverticulum resection. Meckel's diverticulum continues to grow until the age of 3. With this growth, the heterotopic mucosa is displaced distally and moves away from the base of the diverticulum. Bleeding is the main symptom in patients with HM reaching the base of the diverticulum. In patients with bleeding or younger than three years of age, simple diverticulectomy may not be considered safe.Level of Evidence: III

Objective: The primary goal in the treatment of corrosive esophagitis (CE) is to control inflammation and scar reactions. L-glutamine (Gln) is beneficial for the integrity of the intestinal mucosal epithelium and is an amino acid that promotes mature collagen growth. This study was designed to demonstrate the positive results of Gln on injury in corrosive esophagitis. Method: Thirty Sprague-Dawley rats were used in the study. They were divided into 3 groups. CE was formed by dripping 20% sodium hydroxide into the distal esophagus in both groups except the control group (n=10). First group (n=9) was left untreated, while the other group (n=9) was fed orally with the addition of 1 g/kg Gln once a day for 21 days. All rats were sacrificed after 3 weeks. Esophagus of treated and other group rats were examined under light microscope to evaluate collagen deposition, histological damage score and stenosis index. Results: Excess submucosal collagen, muscularis mucosal damage, inflammation and ulceration, which are among the histological damage score parameters, were significantly higher in the untreated group than in the Gln group (p=0.005, p=0.015, p=0.001, respectively). The stenosis index was significantly different (p=0.013). The group treated with Gln had inflammation but no ulceration and necrosis. Conclusion: Our experimental animal study suggests that Gln in nutrition reduces damage in the esophageal mucosa, slows down or partially stops the cellular destruction process that causes stenosis.

Background: Burns are a source of pain, which cannot be fully treated with medications. Objectives: This study aims is to test the effectiveness of lavender oil inhalation aromatherapy applied before dressing change on vital signs and pain levels of children with burns. Design: This randomized controlled study was held between May 2018 and May 2019. A total of 108 children who met the inclusion criteria were studied in three groups: Lavender-15 Group inhaled lavender oil for 15 min before dressing (n:36), Lavender-60 Group inhaled lavender oil for 60 min before dressing (n:36), and Control Group inhaled jojoba (placebo) oil for 15 min before dressing (n:36). Baseline pain levels and vital signs of the children were measured before inhalation. Pain levels and vital signs of the children were re-measured at the 1st and 30th minutes after dressing. Results: There was no significant difference between the groups in terms of pain levels (p = 0.750) and vital signs before dressing. In post-dressing measurements, the number of respiration (after 1 min p = 0.000, after 30 min p = 0.000), heart rate (after 1 min p = 0.000, after 30 min p = 0.000), mean arterial blood pressure (after 1 min p = 0.010, after 30 min p = 0.000) and pain levels (after 1 min p = 0.000, after 30 min p = 0.000) were lower in the Lavender groups compared to the placebo group. Discussion: The result of this research reveals that inhalation aromatherapy which applied before dressing in children with burns affects the reduction of pain levels and stabilization of vital signs.

Purpose: Percutaneous internal ring suturing technique (PIRS) is a minimally invasive technique in pediatric inguinal hernia repair. In the present study, a negative effect on testicular blood flow using PIRS technique has been investigated. Methods: Forty male patients were included in the study prospectively. Two groups were formed as conventional open surgery (Group I) and PIRS technique (Group II). The resistive index (RI) value of the testicular artery was measured prospectively by using SMI (superb micro-vascular imaging) software with the color doppler ultrasound technique preoperatively and postoperatively at the first month. Results: Inguinal hernia was present on the left in 35% (n = 14) of the patients and on the right in 65% (n = 26) of the patients. There was no statistically significant difference (p = 0.727) between Group I and II with regard to preoperative RI value (0.66 ± 0.07 vs. 0.66 ± 0.45, respectively). Similarly, there was no statistically significant difference (p = 0.220) between Group I and II with regard to the RI values measured at the postoperative first month (0.58 ± 0.04 vs. 0.60 ± 0.04, respectively). Although the postoperative RI values decreased compared to the preoperative values in both groups, this difference was not statistically significant. (p = 0.447 in Group I, and p = 0.175 in Group II for intragroup comparison). Conclusions: Besides PIRS technique has the advantages provided by all other laparoscopic techniques defined for inguinal hernia repair, there is no significant difference between this technique and conventional open surgery with regard to testicular blood flow. It is an innovative candidate technique instead of the open surgery method besides its additional advantages.

Objective: The aim of this study was to determine the potential, protective effects of amlodipine in an experimental, ischemia-reperfusion (I/R) model in the rabbit small intestine. Materials and Methods: The rabbits were divided into four groups: sham-operated, amlodipine (10 mg/kg) + sham-operated, I/R, and I/R + amlodipine (10 mg/kg) groups. An intestinal I/R model was applied to the rabbits. The superior mesenteric artery was occluded for 1 h with an atraumatic vascular clamp and then was reperfused for 2 h. Animals in the amlodipine and I/R + amlodipine groups received the amlodipine by oral gavage. At the end of the 2-h-reperfusion period, the animals were sacrificed. Results: Pretreatment with amlodipine significantly increased SOD activity and GSH levels to values close to those found in the serum from the I/R group. Rabbits in the I/R group showed high levels of serum MDA. Amlodipine pretreatment significantly reduced the serum MDA levels compared to the I/R group, although the MDA levels in the I/R + amlodipine group were still higher than in the sham-operated group. The I/R damage was ameliorated by amlodipine pretreatment, as evidenced by histopathological analysis. Conclusion: The present study is the first to report an attenuation of I/R-induced intestinal injury by the systemic administration of amlodipine.

We investigated the potential protective effects of montelukast (MLK) on cecal ligation and puncture (CLP)–induced tissue injury in vital organs — liver, heart, kidneys, and especially lungs — through inhibition of the proinflammatory cytokine response and the generation of reactive oxygen species (ROS) in rats. The rat groups were (1) a 10-mg/kg MLK-treated CLP group; (2) a 20-mg/kg MLK-treated CLP group; (3) a 20-mg/kg MLK-treated, sham-operated group; (4) a CLP control group; and (5) a sham-operated control group. MLK treatment significantly decreased proinflammatory (tumor necrosis factor-alpha, interleukin-6) cytokine levels following CLP. The lipid peroxide level increased in the lung, heart, liver, and kidney tissues after CLP-induced sepsis, and myeloperoxidase activity increased in the lung, heart, and liver tissues. MLK attenuated this elevation in all tissues except the kidney, dose dependently. The glutathione levels and superoxide dismutase activity were significantly increased in the lung, liver, and kidney tissues after MLK treatment. MLK treatment after CLP also potentially reduced mortality. The lung and kidney tissues were the most protected by MLK under sepsis conditions. We can suggest that MLK reverses the systemic inflammatory reaction to polymicrobial sepsis and thereby reduces multiple organ failure.

Objective: Annular pancreas is an uncommon cause of duodenal obstruction in children. In this study, the clinical, radiological, and prognostic findings related to this disorder over a 12-year review period were analyzed.Materials and Methods: A retrospective review of 22 patients with annular pancreas who were treated with surgical repair between April 1998 and February 2010 was performed at two different pediatric surgical units. Presenting symptoms, associated anomalies, radiological findings, the type of surgery performed, postoperative outcomes, and complications were analyzed.Results: Twenty-two patients were identified. Thirteen of the 22 patients (59.1%) were born prematurely, 11 patients (50%) had low birth weight, 2 patients (9.1%) had very low birth weight and 1 patient (4.5%) had extremely low birth weight. The mean birth weight was 2285.23±675.12 g. (970-3300). All patients presented with vomiting, which was bilious in nine (40.9%). Nine patients (40.9%) had chromosomal anomalies. Corrective surgery consisted of duodenoduodenostomy in 9 patients (40.9 %), duodenojejunostomy in 9 patients (40.9%), and gastrojejunostomy in 4 patients (18.1%). Fourteen of the 22 patients have survived (63.6%). The causes of death were combinations of sepsis, pneumonia, brain hemorrhage, and cardiac anomaly. Conclusion: Infants with annular pancreas associated with duodenal obstruction were often born prematurely and/or had low birth weights; many had cardiovascular anomalies. Annular pancreas associated with duodenal obstruction correlated strongly with the trisomy 21 karyotype among the chromosomal anomalies, as did duodenal atresia. The oral feeding tolerance time was nearly the same for all patients regardless of the surgical procedure used.

To investigate the rate of colchicine use in the longitudinal follow-up of familial Mediterranean fever (FMF) carriers and identify variables that could predict the necessity of colchicine treatment in this group. The study was conducted in 9 pediatric rheumatology centers. The files of children with MEFV gene carriers were retrospectively reviewed between February 2014 and May 2024. The study included 869 children with a median follow-up duration of 28 months (12-124). In most of the cases (n: 369; 43.5%), MEFV gene analysis was ordered by a pediatric rheumatologist, while in 228 children (26.2%), gene analysis was conducted at the request of a geneticist. The most common reason for ordering MEFV gene analysis was the presence of FMF-like symptoms (n: 349; 40.1%), followed by genetic screening due to a family history of FMF in relatives (n: 267; 30.7%). Colchicine therapy was initiated in 13.9% (n: 121) of the children. Variables that showed statistically significant differences in colchicine users included having a family history of amyloidosis, the MEFV gene ordered by a pediatric rheumatologist, and the presence of FMF-like symptoms. Conclusions: A small number of MEFV gene carriers develop FMF symptoms during the follow-up period, most commonly within 2-3 years. We do not recommend routine family screening for the MEFV gene after the diagnosis of an index patient unless there is a history of amyloidosis in the family or individuals having FMF-like symptoms., Competing Interests: Declarations. Ethics approval: The study was approved by the local ethics committee of Zeynep Kamil Women and Children’s Diseases Training and Research Hospital (date: 20 March 2024, number: 38) and was performed according to the tenets of the Declaration of Helsinki. Written informed consent was received from the legal guardians of the children. Consent to participate: Informed consent was obtained from the legal guardians of the children. Consent for publication: Informed consent for publication was obtained from the legal guardians of the children. Competing interests: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Proper nutrition is a significant contributor to growth achievement in patients with juvenile idiopathic arthritis (JIA). In this study, the aim was to analyze the growth parameters and nutritional status of children with JIA and then compare them with their healthy peers., Methods: A cross-sectional study was conducted with 54 patients with JIA and the same number of healthy peers. Growth parameter z-scores and nutrient distributions were analyzed and compared with a control group and among disease subgroups., Results: While the average height in the control group was significantly greater than in the patient group, there was similarity in terms of body weight and body mass index (BMI) (P < 0.001, P = 0.33, P = 0.14, respectively). Body weight and BMI z-scores of patients with high disease activity at the most recent visit were significantly lower (P = 0.03, P = 0.01, respectively). Both groups had similar energy and protein requirement-meeting percentages (P = 0.62, P = 0.51). JIA atients had higher carbohydrate intake (P = 0.04), and fat intake was higher in controls (P = 0.02). Energy obtained from junk food was higher in patients with entesitis-related arthritis (ERA) compared to oligoarticular JIA and polyarticular JIA (P = 0.03). Micronutrient intake in the ERA group was significantly lower for vitamin E, C, and folate (P = 0.02, P = 0.03, P < 0.001)., Conclusion: In our cohort, patients had a lower height score. As they have a diet characterized by adequate energy/protein, carbohydrate, and high fat intake, this may be a reflection of disease activity. Although some of the micronutrient intakes were less than normal in both groups, significant deficiencies were identified in the ERA group., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

To evaluate the sleep quality and fatigue levels in children with familial Mediterranean fever (FMF) in comparison to healthy children. The Pediatric Quality of Life Multidimensional Fatigue Scale (PedsQL-MFS) and the Pittsburgh Sleep Quality Index (PSQI) were the instruments utilized to assess fatigue and sleep quality in children with FMF and controls, respectively. Spearman's rank coefficient was decisive in determining the association between patient-reported outcome measures and disease-related features. Two hundred twenty-five (59.3% female) patients and 182 (51.6% female) healthy counterparts were enrolled in the study. In PSQI, where high scores indicate sleep disturbance, the median score was significantly higher in the patient group (5; 3-6) than the control group (3; 2-4) (p < 0.001). PEDsQL-MFS demonstrated significantly lower fatigue levels in the control group than patients (p = 0.01). The level of fatigue in the patient group was found to increase in correlation with sleep problems (r: - 0.750, p < 0.001). Additionally, a high correlation was present between the PSQI/PedsQL-MFS scores and the number of attacks in the last year (r: - 0.645, p < 0.001/r: 0.721, p < 0.001, respectively). There was no difference in terms of fatigue and sleep disorders between mutations (homozygous, heterozygous, or compound heterozygous) in the MEFV gene (p > 0.05).    Conclusion: High disease activity has a significant negative impact on the sleep quality and fatigue levels of patients with FMF. This study emphasizes the importance of assessing fatigue and sleep quality with objective outcome tools periodically in FMF patients throughout the disease course. What is Known: • Fatigue is a common matter that often accompanies rheumatic diseases and causes disability. • Chronic rheumatic diseases often experience poor sleep quality. What is New: • In high correlation with the disease severity of familial Mediterranean fever, sleep quality decreases and fatigue level increases significantly. • In familial Mediterranean fever patients, a negative correlation is present between age and the general fatigue and sleep/rest related fatigue scores (low scores indicating greater fatigue) and sleep quality is poorer in the adolescent age group., (© 2024. The Author(s).)

Objectives: Colchicine forms the mainstay of treatment in FMF. Approximately 5-10% of FMF patients are colchicine resistant and require anti-IL-1 drugs. We aimed to compare the characteristics of colchicine-resistant and colchicine-responsive patients and to develop a score for predicting colchicine resistance at the time of FMF diagnosis., Methods: FMF patients (0-18 years) enrolled in the Turkish Paediatric Autoinflammatory Diseases (TURPAID) registry were included. The predictive score for colchicine resistance was developed by using univariate/multivariate regression and receiver operating characteristics analyses., Results: A total of 3445 FMF patients [256 (7.4%) colchicine-resistant and 3189 colchicine-responsive) were included (female:male ratio 1.02; median age at diagnosis 67.4 months). Colchicine-resistant patients had longer, more frequent attacks and were younger at symptom onset and diagnosis (P < 0.05). Fever, erysipelas-like erythema, arthralgia, arthritis, myalgia, abdominal pain, diarrhoea, chest pain, comorbidities, parental consanguinity and homozygosity/compound heterozygosity for exon 10 MEFV mutations were significantly more prevalent among colchicine-resistant than colchicine-responsive patients (P < 0.05). Multivariate logistic regression analysis in the training cohort (n = 2684) showed that age at symptom onset, attack frequency, arthritis, chest pain and having two exon 10 mutations were the strongest predictors of colchicine resistance. The score including these items had a sensitivity of 81.3% and a specificity of 49.1%. In the validation cohort (n = 671), its sensitivity was 93.5% and specificity was 53.8%., Conclusion: We developed a clinician-friendly and practical predictive score that could help us identify FMF patients with a greater risk of colchicine resistance and tailor disease management individually at the time of diagnosis., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Objective: To reveal the vaccination status of patients with pediatric rheumatic disease (PedRD) and to compare this with healthy controls., Methods: The electronic health records of the Ministry of Health regarding the vaccination status of children with PedRD followed in a tertiary hospital were analyzed cross-sectionally and compared with their healthy controls. The missing vaccines were reported according to individual, age-appropriate schedule and causes of skipped vaccines in both groups were investigated with an online survey., Results: The vaccination rate of patients in the last examination was 71.4% (90/126) and 95.7% (110/115) in healthy controls ( p  < 0.001). Measles-mumps-rubella vaccine, diphtheria, the administration rates of the second dose of tetanus-acellular pertussis-inactivated polio and Haemophilus influenzae type B, chickenpox, and hepatitis A vaccines were significantly lower in patients than in controls ( p values 0.004, 0.02, 0.01, 0.013, respectively). The pre-diagnosis incomplete vaccination proportion was significantly higher in the patient group (16.6%) than in healthy controls (4.3%) ( p  = 0.002). In the patient group, the proportion of incomplete live-attenuated vaccines after diagnosis (25%) was more than pre-diagnosis (61.1%) ( p  = 0.04), while the proportion of incomplete non-live vaccines before and after diagnosis was similar (47.2% and 50%, respectively) ( p  = 0.73). The major reasons for missed vaccines were physicians' recommendations (15.6%), the presence of PedRD diagnosis (12.5%), and the drugs used (12.5%)., Conclusion: Vaccination coverage of PedRD patients has been shown to lag behind the routine vaccination schedule (71.4%). In addition to new recommendations, electronic health system records for vaccination may be appropriate for the follow-up of these patients, and the addition of reminder alerts may be useful to reduce the rate of missed vaccinations.

Objectives: The rapid expansion in the use of telemedicine after the COVID-19 pandemic has led many patients with chronic diseases to seek alternative ways for follow-ups. This study aimed to investigate the demands and opinions of parents of children with rheumatic diseases toward telemedicine and to examine the factors affecting telemedicine preference. Methods: A single-center, cross-sectional, Web-based survey study was conducted. Sociodemographic data, characteristics of the disease, access to the clinic, internet use, and views on telemedicine were assessed. Factors effecting telemedicine preference were evaluated by multivariate analysis. Results: A total of 245 parents have completed the survey. The diagnoses of patients were recurrent fever syndromes (55.1%), juvenile idiopathic arthritis (31.0%), systemic connective tissue diseases (8.2%), and vasculitis (5.7%). The majority of patients came to the clinic by public transport ( n  = 190, 77.6%). Sixty-eight (27.8%) patients missed at least one outpatient appointment in the last year. Majority ( n  = 172, 70.2%) of parents stated that they would prefer telemedicine visits if it becomes available. Multivariate analysis revealed that the most related factors to telemedicine preference were higher education level (odds ratio [OR]: 6.69, confidence interval [95% CI]: 2.21-20.25, p  = 0.001), missing an appointment (OR: 3.04, 95% CI: 1.41-6.56, p  = 0.004), and travel time longer than 1 h (OR: 2.13, 95% CI: 1.13-3.86, p  = 0.012). Conclusion: Telemedicine visits are in demand in pediatric rheumatology and should be considered an alternative method to ensure continuity of patient follow-up. A personal approach should be followed when selecting patients for telemedicine.

Classical attacks of familial Mediterranean fever (FMF) are often accompanied by fever, but some of the patients have attacks without fever. This study aimed to compare the characteristics of FMF patients with and without fever during their attacks and draw attention to the different clinical presentations of FMF in children. Medical files of patients aged 0-18 years who were followed up with the diagnosis of FMF in two reference pediatric rheumatology centers were reviewed retrospectively. The patients were divided into two groups: children who had had no fever in any of their attacks were assigned as group 1, and those who had fever during their attacks were classified as group 2. Out of 2003 patients evaluated, 191 (9.53%) patients had attacks not accompanied by fever and their median age at onset of symptoms (7.0 vs. 4.0 years, p < 0.001) and the median age at diagnosis (8.6 vs. 6.0 years, p < 0.001) were significantly higher; however, group 2 had a delay in diagnosis. The annual number of attacks and abdominal attacks were more common in group 2; arthritis, arthralgia, erysipelas-like rash, exercise-induced leg pain, and myalgia were more common in group 1.    Conclusion: The data from the assessment of children with FMF attacks not accompanied with fever were presented for the first time. Children with late age onset of FMF and dominance of musculoskeletal features may display attacks not accompanied with fever. What is Known: • Familial Mediterranean fever (FMF) is the most common inherited auto-inflammatory disease, characterized by recurrent attacks of fever, serositis, and musculoskeletal symptoms. • Although fever is the most common symptom, few studies have reported attacks without fever. What is New: • The aim of this study was to identify patients with FMF but without fever during attacks and to demonstrate their distinctive presentations. • We found that 7% of our patients had afebrile attacks with predominant musculoskeletal symptoms and were diagnosed earlier than patients with febrile attacks, probably due to early referral to pediatric rheumatology clinics., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Rheumatoid factor (RF)-positive polyarthritis is the least common type of juvenile idiopathic arthritis (JIA). Functional disability in RF-positive polyarthritis patients is much more severe than in patients with other subtypes; but data on this subtype alone is limited. This study aimed to analyze clinical features, long-term follow-up, treatment response, and remission status in a large multicenter cohort of RF-positive polyarthritis patients., Methods: This retrospective study included RF-positive polyarthritis patients that were followed up for ≥ 6 months between 2017 and 2022 by the Pediatric Rheumatology Academy (PeRA)-Research Group (RG). Data on patient demographics, clinical and laboratory characteristics were obtained from medical charts. JIA treatments and duration of treatment were also recorded. The patients were divided into 2 groups based on methotrexate (MTX) response, as follows: group 1: MTX responsive, group 2: MTX unresponsive. Clinical and laboratory findings were compared between the 2 groups., Results: The study included 56 (45 female and 11 male) patients. The median age at onset of RF-positive polyarthritis was 13.2 years [(interquartile range) (IQR): 9.0-15.0 years] and the median duration of follow-up was 41.5 months (IQR: 19.5-75.7 months). Symmetrical arthritis affecting the metacarpophalangeal and proximal interphalangeal joints of the hands was commonly observed. Subcutaneous MTX was the preferred initial treatment; however, it was ineffective in 39 (69.6%) of the patients. Of 25 patients followed for 24 months, 56% still had active disease at 24 months., Conclusion: During 2 years of treatment, 44% of RF-positive polyarthritis patients have inactive disease, and they should be considered as a distinct and important clinical entity requiring aggressive and early treatment., (© 2023. The Author(s).)

Objective: To evaluate the safety of canakinumab using real-world data in patients with systemic juvenile idiopathic arthritis (sJIA) and autoinflammatory diseases (AID)., Research Design and Methods: This was a cross-sectional observational, multicenter study. Patients diagnosed with AID and sJIA treated with canakinumab were included in the study. The participating 13 centers retrospectively collected their patients' data., Results: A total of 335 patients were involved in the study. Among these patients, 280 were in the AID group and 55 were in the sJIA group. Canakinumab was administered at a median dose of 3 (2.5-4) mg/kg. The median total exposure time to canakinumab was 1.9 (0.8-3.2) years, corresponding to 759.5 patient-years. Seven hundred and seventy-nine total adverse events (AE) were identified. The total incidence of AE, and serious adverse events (SAE) throughout the study period was 1.02 per patient-years. The upper respiratory tract infection rate was 0.7 per patient-years, while the other infection rate was 0.13 per patient-years. While no death was observed in any patient, SAE were observed in 8 patients. Interstitial lung disease, anaphylaxis, or anaphylactoid reactions were not observed in any patient., Conclusions: Real-life data from a large cohort of patients suggests that canakinumab is as safe as claimed in clinical trials.

Objectives: Behçet's disease (BD) is a systemic vasculitis affecting many organ systems, with the involvement of all-sized arteries and veins. The study aims to determine the main characteristics of paediatric BD patients and also analyse the clustering phenotypes., Methods: Demographic data, clinical manifestations, laboratory features, treatment schedules, and disease outcomes were achieved from patients' charts retrospectively. A cluster analysis was performed according to the phenotype., Results: A total of 225 (109 male/116 female) patients with BD were enrolled in the study. The median ages of disease onset and diagnosis were 131 (36-151) and 156 (36-192) months, respectively. According to cluster analysis, 132 (58.6%) patients belonged to the mucocutaneous-only cluster (C1), while 35 (15.6%) patients fitted to articular type (C2), 25 (11.1%) were in the ocular cluster (C3), 26 (11.6%) were in the vascular cluster (C4), and 7(3.1%) belonged to the gastrointestinal cluster (C5). Ocular and vascular clusters were more common in boys (p < .001), while girls usually presented with the mucocutaneous-only cluster. The disease activity at the diagnosis and the last control was higher in ocular, vascular, and gastrointestinal clusters., Conclusions: These identified juvenile BD clusters express different phenotypes with different outcomes Our analysis may help clinicians to identify the disease subtypes accurately and to arrange personalized treatment., (© Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: To investigate patients who flared after discontinuation of biological disease-modifying anti-rheumatic agents (bDMARDs) and identify risk factors associated with flare., Methods: A multicenter study evaluating systemic and non-systemic juvenile idiopathic arthritis (sJIA and non-sJIA) patients whose bDMARDs were ceased after remission., Results: A total of 101 patients whose bDMARDs were ceased after remission was evaluated. Children with sJIA had the lowest risk of flare and 11.1% of 36 sJIA patients experienced flare after a median of 9 (4-24) months of bDMARDs cessation with three of them flaring in the first year. High leukocyte counts in sJIA patients were associated with inactive disease at 1-year after the start of treatment (p = 0.004). In the non-sJIA group, 46.1% patients experienced flare after a median of 7 (1-32) months of biologic cessation, and of these, 25 flared in the first year. Antinuclear antibody positivity (p = 0.02), earlier disease onset (p = 0.03), long disease duration (p = 0.01), and follow-up (p = 0.02) and extended time from diagnosis to first biological onset (p = 0.03) were more common among patients with flare., Conclusions: When considering discontinuation of bDMARDs, it should be kept in mind that the risk of exacerbation requiring re-initiation therapy is quite significant within the first year after discontinuation of therapy.

To evaluate the safety profile of measles, mumps and rubella (MMR) booster in children diagnosed with rheumatic diseases receiving biological agents. The study included retrospective safety data of children administered MMR booster dose receiving biologics or biologics with methotrexate. The files of 182 patients were accessed from the pediatric rheumatology biological therapy archive, and the vaccination status of these children was obtained by accessing electronic records. Of 182 patients, 14 patients were vaccinated with MMR booster dose. Thirteen of the patients were followed up with a diagnosis of juvenile idiopathic arthritis and one with colchicine-resistant familial Mediterranean fever. None of the patients had disease exacerbation after vaccination, and three patients had mild side effects consisting of rash, angioedema, joint pain, and fatigue.    Conclusion: This study supports the data regarding evidence of the safety of MMR booster dose administration in children with rheumatic diseases receiving bDMARDs. What is Known: • MMR booster is avoided in immunocompromised pediatric patients receiving bDMARDs except in specific conditions. What is New: • The MMR booster dose may be safe in children with PedRD receiving bDMARDs or bDMARDs with MTX. These bullets can be added to the manuscript., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: The coronavirus disease 2019 (COVID-19) vaccine represents a cornerstone in tackling the pandemic and with the approval of the BNT162b2 mRNA vaccine in December 2020, it has become a beacon of hope for people around the world, including children. This study aimed to present the data on the humoral response and safety of vaccine in a cohort of patients with paediatric rheumatic diseases receiving immunomodulatory treatments., Methods: Forty-one children with paediatric rheumatic diseases were included and were vaccinated with the BNT162b2 mRNA vaccine (two doses of 30 µg administered 3-4 weeks apart). To assess the humoral response, IgG antibodies developed against the S1/Receptor-binding domain (RBD) of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike protein at baseline and 3-4 weeks after the second dose were measured. The possible local and systemic side effects and disease activity scores were evaluated during the study period., Results: After the second dose of vaccine, markedly elevated anti-RBD IgG titres were observed in all patients with a median titre of 20 474 AU/ml [interquartile range (IQR) 6534-36 151] with a good safety profile. The median disease duration was 4.3 (IQR 3.5-5.6) years. In the cohort, 14 (34.1%) received conventional DMARDs (cDMARDs), 16 (39%) received biologic DMARDs (bDMARDs) and 11 (26.8%) received a combined therapy (cDMARDs and bDMARDs). Patients treated with combined therapy [median 4695 (IQR 2764-26 491)] had significantly lower median titres of anti-RBD IgG than those receiving only cDMARDs., Conclusion: Paediatric rheumatic diseases patients receiving immunomodulatory treatments were able to mount an effective humoral response after two dose regimens of BNT162b2 mRNA vaccine safely without interrupting their current treatments., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Biosimilars have been adopted by clinicians more slowly than anticipated in the post-marketing phase., Objectives: We aimed to reveal the perceptions and attitudes of pediatric rheumatologists towards biosimilars and the obstacles to biosimilar therapy., Methods: A web-based survey designed to determine the knowledge, experience, and perceptions of pediatric rheumatologists about biosimilars was electronically mailed to the participants between April and August 2021. Responses were collected anonymously and subsequently analyzed., Results: A total of 114 pediatric rheumatologists including fellows (32.4%), specialists (29.8%), and seniors (37.7%) responded to the questionnaire. According to the data, 75 (65.8%) physicians had already prescribed at least one biosimilar. The vast majority of participants were aware of the potential cost savings of biosimilars (84, 73.3%). Participants who felt insufficiently informed were 41.8%, 67.6%, and 83.7% among seniors, specialists, and fellows, respectively. In pediatric rheumatology, the scarcity of clinical trials and real-life data (64%) and inadequate information about tolerance to the biosimilars and related side effects in children (49.1%) were the most common barriers expressed by prescribers. Nearly half (45%) of the pediatric rheumatologists preferred to prescribe biosimilars in the treatment of biologic-naive cases. However, most (93%) were reluctant to switch a reference molecule to a biosimilar while the patient was doing well under the originator medicine., Conclusions: This survey provided insights into the concerns about prescribing biosimilars among pediatric rheumatologists. In the field of pediatric rheumatology, further education about biosimilars and real-life experiences is required to better inform about treatment options in children., (© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Background: Vaccination programs are effective strategies in preventing infectious diseases and controlling epidemics. Vaccination against SARS-CoV-2 in children has not yet been approved globally, and it is unclear what attitude families will take when it is approved in children. We aimed to investigate the underlying causes of vaccine acceptance, hesitation, and refusal, as well as concerns about the acceptability of the COVID-19 vaccine by parents of children with rheumatic diseases., Methods: Parents of children followed up with a diagnosis of rheumatic disease in the pediatric rheumatology outpatient clinic of a university hospital were included in the study. We applied a closed web-based online survey conducted cross-sectionally and sent to the participants via mobile smartphones., Results: For fathers, mothers, and their children, acceptance rates for a COVID-19 vaccine were 64.2%, 57.7%, and 41.8%, respectively. In the multivariate analysis, factors affecting parents' acceptance of vaccines for their children were as follows: "Receiving antirheumatic medications regularly (AOR 5.40, 95% CI 1.10-26.33, p = 0.03), the previous history of getting special recommended vaccines (AOR 4.12, 95% CI 1.12-27.85, p = 0.03), relying on vaccines for ending pandemic (AOR 8.84, 95% CI 2.80-27.85, p = 0.001), complying with the pandemic measures entirely (AOR 5.24, 95% CI 1.46-18.74, p = 0.01)". The two most common reasons for vaccine rejection were fear of the side effects of the vaccine and its possible interaction with rheumatic drugs used by children., Conclusion: According to our survey, parents were more likely to accept a COVID-19 vaccine for themselves than their children. The success of COVID-19 vaccination programs sources highly on people's willingness to accept the vaccine. It is crucial to vaccinate children for achieving herd immunity and in terms of avoiding vaccine hesitancy. Larger data examining the causes of concerns in parents of both healthy children and children with chronic diseases should be delineated., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Vitamin D has an immunomodulating property that regulates the inflammatory response. In this study, the aim was to evaluate the relationship between vitamin D levels and clinical severity and inflammation markers in children and adolescents with COVID-19. The clinical and laboratory records of 103 pediatric cases with COVID-19, whose vitamin D levels had been measured, were retrospectively reviewed. The cases were divided into groups according to their clinical severity (asymptomatic, mild, and moderate-to-severe) and vitamin D levels. The moderate-to-severe clinical group had significantly higher inflammation markers (CRP, procalcitonin, fibrinogen, D-dimer) and a lower lymphocyte count compared to both the mild and asymptomatic groups. The 25 OH vitamin D levels were also significantly lower (p < 0.001), and the ratio of vitamin D deficiency was 70.6% in the moderate-to-severe group. The vitamin D-deficient group had a significantly higher age and fibrinogen levels while also having a lower lymphocyte count compared to the insufficient and normal groups. The 25 OH vitamin D level was correlated positively with the lymphocyte count (r = 0.375, p = <0.001), and negatively with age (r = -0.496, p = <0.001), CRP (r = -0.309, p = 0.002) and fibrinogen levels (r = -0.381, p = <0.001). In a logistic regression analysis, vitamin D deficiency, D-dimer, and fibrinogen levels on admission were independent predictors of severe clinical course.Conclusion: This study revealed an association between vitamin D deficiency and clinical severity, in addition to inflammation markers in pediatric COVID-19 cases. Prophylactic vitamin D supplementation may be considered, especially in the adolescent age group. What is Known: • • The pathology of COVID-19 involves a complex interaction between the SARS-CoV-2 and the immune system. Hyperinflammation/cytokine storm is held responsible for the severity of the disease. • Vitamin D has multiple roles in the immune system that can modulate the body reaction to an infection. What is New: • • Clinically more severe group had significantly lower vit D levels and significantly higher inflammation markers. • Lower 25 OH vit D levels were associated with higher inflammation markers, suggesting an important role of vitamin D in the clinical course of COVID-19 in children and adolescents probably by regulating the systemic inflammatory response., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Environmental tobacco smoke (ETS) exposure is associated with many diseases, including lower respiratory tract infections, in pediatric age groups. In this study, we aimed to assess the effect of household ETS exposure on symptom frequency and clinical severity in children and adolescents with COVID-19., Materials and Methods: This retrospective study included pediatric cases (<18 years old) with a positive SARS-CoV-2 test, who were admitted to our hospital between 20.03.2020 and 01.05.2020. Patients with respiratory diseases and active smokers were excluded. Demographic characteristics, symptoms, and clinical severity of COVID-19 were obtained from parents and children using a questionnaire and from patients' files. Household ETS exposure was assessed by questionnaire., Results: A total of 167 patients (median age 145 months, 50.7 % male) were included in the study. The frequency of household ETS exposure was 50.9%, and the frequency of cough was significantly higher in exposed children than non-exposed children (71.4% vs 50.8% respectively, p=0.02). The frequency of both fever and cough was significantly higher ETS-exposed in the subgroup of cases with a mild clinical course (69.5% vs 48.1% respectively, p= 0.02 for fever and 67.8% vs 44.4% respectively, p=0.01 for cough). There was no relationship between clinical severity and household ETS exposure., Conclusion: Household ETS exposure may impact COVID-19 pediatric cases as demonstrated by the increased frequency of cough and fever in ETS-exposed children. As new lockdown measures are implemented, increasing public awareness about the effect of household ETS exposure on COVID-19as well as encouraging a decrease in ETS exposure are essential., Competing Interests: Conflict of Interest: The authors have no conflict of interest to declare., (© Copyright 2021 by The Turkish Archives of Pediatrics.)

Background: As an important feature in patient-physician communication for both primary and clinical care, empathy is one of the basic competencies that physicians should possess. The primary aim of this study was to evaluate the level of empathy among medical students in all years of medical training using two different instruments: the Jefferson Scale of Physician Empathy (for clinical empathy level) and the Toronto Empathy Questionnaire (for general empathy level)., Materials and Methods: This study is a cross-sectional descriptive study conducted in 2017-2018 academic year with students studying at Akdeniz University Faculty of Medicine. Data collection form, Toronto Empathy Questionnaire (TEQ) and Jefferson Scale of Physician Empathy (JSPE) was applied to the students by the researchers. The statistical analysis was carried out by using IBM-SPSS version 23 for Mac OS. T-test, ANOVA test, Spearman and Pearson correlation analysis were used for comparisons., Results: The mean TEQ score of the students was 52.8/65 and the JSPE-S score was 80.3/100. TEQ scores of students increased up to 4th year and then decreased, but the difference between the years was not statistically significant. The third year students' JSPE-S scores were significantly higher than that of the sixth year students., Conclusion: While the clinical empathy levels of medical students decreased significantly after 3rd year, the general empathy levels decreased less. This result shows us that we should review our medical education curriculum and educational environment, and should initiate initiatives, and devote more time to empathy education in order to prevent the decrease in empathy level and increase empathy during medical education., Competing Interests: Declaration of conflicting interests:The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2020.)