Search

Background Nailfold videocapillaroscopy (NVC) is the primary diagnostic tool for the assessment of microcirculation in the pediatric population. Objective To define and standardize age-specific normal NVC patterns in healthy children and adolescents. Methods A cross-sectional observational multicentric study was conducted in 564 participants aged 5–17 years. Dino-Lite CapillaryScope 200 Pro Model MEDL4N Pro was performed at 200× magnification. Quantitative and qualitative NVC parameters were analysed separately for each age group and divided into four groups based on age categories. Results Of the 564 healthy participants, 54.9% were female. A total of 1184 images and 3384 capillaries were analysed. Positive correlations were observed between age and capillary density (P  < 0.001, R = 0.450, CI95% 0.398–0.503). There was also a positive correlation between age and arterial/venous, loop diameter and capillary length, whereas there was a weak negative correlation between intercapillary distance. However, no correlation was found between age and capillary width. In addition, capillary density was significantly lower in the 5–7 age group compared with the other patient groups. Arterial limb diameter was lower in the 5–7 age group, while venous limb diameter was significantly wider in the 15–17 age group compared with the other patient groups. Dilated capillaries (8.7%), capillary tortuosity (14.4%), crossed capillaries (43.1%), micro-haemorrhages (2.7%) and avascular area (4.8%) were present in all age groups. Excellent intra- and interobserver ICC values were obtained for all parameters. Conclusion These findings hold potential significance for future studies, aiding in the analysis and differentiation of children suspected of rheumatological diseases with potential microangiopathy. [ABSTRACT FROM AUTHOR]

Objectives To describe the clinical features and treatment outcomes of children with juvenile psoriatic arthritis (JPsA) and compare the distinct patterns of the disease between early-onset and late-onset age groups. Methods Patients with JPsA followed regularly for at least 6 months between 2010 and 2020 in seven paediatric rheumatology centres in Turkey were included in the study. The demographic features, clinical manifestations, treatment strategies and outcomes of the patients were evaluated retrospectively. Results A total of 87 (46 male/41 female) patients were included in the study. The mean age at diagnosis was 11.9 years (s. d. 4.5). Fifty-seven (65.5%) patients had psoriasis at the time of diagnosis and arthritis preceded psoriasis in 10 (11.5%) patients. Thirty (34.5%) patients had dactylitis, 28 (32.2%) had nail pitting, 36 (41.4%) had involvement of the small joints and 20 (23%) had enthesitis. Sacroiliitis was detected in 11 (12.6%) patients by MRI. ANA was positive in 35 (40.2%) patients. Twelve children (13.8%) were in the early-onset (<5 years) group. Uveitis and ANA positivity were more common in the early-onset group. Active joint counts and activity scores of our patients showed significant improvement at month 6 and at the last control compared with baseline. Conclusion About one-third of patients with JPsA do not have psoriasis at the time of diagnosis. In some patients, no skin lesion is seen during the course of the disease. Children with PsA seem to display two different phenotypes. Younger children have a female predominance, ANA positivity and uveitis, while older children have more axial involvement. [ABSTRACT FROM AUTHOR]

Objectives We aimed to comprehensively analyse the available literature to identify the unmet requirements in transitional programs tailored specifically for patients diagnosed with JIA. Methods According to published guidance on narrative reviews, a systematic review of the literature on transitional care in rheumatology was conducted. Pertinent documents were collected from reputable databases, such as Web of Science, Scopus, and MEDLINE/PubMed. The search encompassed literature published from the inception of each database until January 2023. Results In this study, a comprehensive analysis of the findings of 34 studies was conducted. Among these, 12 studies focused on assessing the readiness of adolescents and young adults diagnosed with JIA. Additionally, 18 studies examined the effectiveness of structured transition programs in terms of adherence and satisfaction. Finally, 4 studies investigated disease-related outcomes in this population. Conclusion The need for transitioning children with rheumatic diseases to adult rheumatology services for continued care is clearly evident. However, the absence of established best practice guidelines presents a challenge in facilitating this transition effectively. Although several scoring systems have been proposed to ensure organized and seamless transfers, a consensus has not yet been reached. Furthermore, the socio-economic and cultural variations across countries further complicate the development of universal guidelines for transitioning children with rheumatic diseases. To address these concerns, our objective in conducting this literature review was to emphasize the significance of this issue and identify the specific requirements based on the unmet needs in the transition process. [ABSTRACT FROM AUTHOR]

Background: The aim of the study was to evaluate the approaches of pediatric rheumatologists and pediatric hematologists to patients with similar musculoskeletal (MSK) complaints and to highlight the differences that general pediatricians should consider when referring patients to these specialties. Methods: This is a cross-sectional study involving the patients who applied to pediatric rheumatology centers with MSK complaints and were diagnosed with malignancy, as well as patients who were followed up in pediatric hematology centers with a malignancy diagnosis, and had MSK complaints at the time of admission. Results: A total of 142 patients were enrolled in the study. Of these patients, 83 (58.4%) applied to pediatric rheumatology centers, and 59 (41.6%) applied to pediatric hematology centers. Acute lymphoblastic leukemia (ALL) was the most common diagnosis among the patients who applied to both centers, with 80 cases (56.3%). The median age of diagnosis was 87 (interquartile range, IQR: 48-140) months. The most common preliminary diagnosis in pediatric rheumatology centers was juvenile idiopathic arthritis (JIA), with 37 cases (44.5%). MSK involvement was mainly seen as arthralgia, and bone pain. While arthralgia (92.7%) was the most common complaint in rheumatology centers, bone pain (88.1%) was more common in hematology centers. The most frequently involved joints were the knee (62.9%), ankle (25.9%), hip (25%), and wrist (14%). The most common laboratory abnormalities were high lactate dehydrogenase (LDH), high C-reactive protein (CRP), anemia, and high erythrocyte sedimentation rate (ESR). Thrombocytopenia, neutropenia, and high LDH were statistically significantly more frequent in patients admitted to hematology centers than in patients admitted to rheumatology centers (p<0.001, p=0.014, p=0.028, respectively). Patients who applied to rheumatology clinics were found to have statistically significantly higher CRP levels (p=0.032). Conclusions. Malignancies may present with only MSK system complaints in childhood. Therefore, malignancies should be included in the differential diagnosis of patients presenting with MSK complaints. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: To investigate the single-center experience of the efficacy and safety profile of biologic therapies in patients with juvenile idiopathic arthritis (JIA) and identify risk factors associated with adverse events (AEs). Methods: The medical charts of children with JIA diagnosed between January 2010 and December 2021 were reviewed retrospectively, and patients treated with biological agents were included in the study. Demographic data, clinical features, laboratory results, treatments used, and AEs during the treatment period were collected. Results: From the total JIA cohort (n=814), 237 patients who received biologic therapy for at least 3 months were enrolled in the study. The most frequent subtype was persistent oligoarticular JIA (45.1%) and the most frequently used biologic drug was etanercept (n=118), followed by adalimumab (n=64), tocilizumab (n=31), anti-interleukin-1 (anti-IL-1) agents (n=12; 7 anakinra and 5 canakinumab), infliximab (n=6), abatacept (n=3), secukinumab (n=2) and tofacitinib (n=1). One hundred sixty-four (69.2%) patients received disease-modifying antirheumatic drugs (DMARDs) concomitantly, 10.5% received DMARDs plus corticosteroids and 2.1% received only corticosteroids. The median [interquartile range (IQR)] age and median age at initiation of the biologics were 14.4 (10.7-18) years and 10.9 (6.6-14.5) years, respectively. The median (IQR) follow-up period was 3.9 (2-6.3) years. On biologic therapy, the median (IQR) JADAS-71 decreased from 13 (11-19) at baseline to 0 (0-2) after median 22 (10-40) months of treatment (p<0.001). The most frequent AE was local injection site reactions with biologics administered subcutaneously (n=8), followed by upper respiratory tract infections (n=4) and diffuse erythematous skin rashes (n=4). Serious AEs were observed in 11 (4.6%) patients. To compare the frequency of AEs, patients were divided into three groups according to the biologics administered, as follows: Group 1: Tumor necrosis factor inhibitors, group 2: anti-IL-1 agents, group 3: anti-IL-6 agent. The frequency of AEs was significantly higher in JIA patients on anti-IL-1 therapy than in the other two groups (58.3% vs. 29% and 8.5%, p<0.001). Conclusion: Biological agents are used with increasing frequency in children with JIA, and their off-label use is quite common. Although these agents are considerably effective and quite safe, AEs should not be underestimated. While planning the management of patients with refractory JIA, careful interpretation of benefit-risk balance for every individual patient seems to be reasonable and required. [ABSTRACT FROM AUTHOR]

Aim: The Juvenile Spondyloarthritis Disease Activity Index (JSpADA) is the only disease activity score specifically validated for children with enthesitis-associated arthritis (ERA). It was developed to address the need for an effective measurement tool to assess disease activity in this population. We aimed to evaluate the clinical course of patients with ERA using JSpADA and to compare the effects of treatment modalities using JSpADA. Methods: This cross-sectional observational study enrolled 61 patients with ERA who were followed up between January 2020 and 2023. Clinical features, treatment options, and JSpADA were noted in electronic medical files. The effectiveness of treatment modalities was compared by JSpADA. Results: The median age of onset of the group was 10 [interquartile range (IQR), 9-15] years. The study cohort included three groups of patients: 1) DMARD received (n=34); 2) biologic drug received (n=14); 3) DMARD and biological combination received (n=13). Forty-three cases (70%) presented with peripheral arthritis, including enthesitis, whereas 18 (30%) patients had axial involvement. At disease onset, the median JSpADA scores were 2 (IQR, 2-3), 2.5 (IQR, 2-3), and 3.5 (IQR, 2.5-5) in groups 1, 2, and 3, respectively (p=0.27). At the first year of follow-up, there was a significant improvement in the disease activity of groups 1 and 2 (p=0.02 and p=0.04). However, there was no significant reduction in JSpADA values in the third group. Conclusion: In patients with ERA, intermittent JSpADA evaluation during visits can guide the objective and accurate follow-up and treatment response of patients. [ABSTRACT FROM AUTHOR]

Copyright of Medical Journal of Bakirkoy is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

OBJECTIVE: This study aimed to look for the initial manifestations of juvenile dermatomyositis (JDM), give follow-up results, and search for risk factors for the development of calcinosis. METHODS: The files of children with JDM diagnosed between 2005 and 2020 were reviewed retrospectively. RESULTS: The study included 48 children, 33 girls and 15 boys. The mean age at the onset of the disease was 7.6±3.6 years. The median duration of follow-up was 35 (6-144) months. Twenty-nine patients (60.4%) had monocyclic, 7 (14.6%) patients had polycyclic, and 12 (25%) patients had chronic persistent disease course. At the time of enrollment, 35 (72.9%) patients were in remission, while 13 (27.1%) patients had active disease. Calcinosis developed in 11 patients (22.9%). Children having myalgia, livedo racemosa, skin hypopigmentation, lower alanine aminotransferase (ALT) levels, and higher physician visual analog scores at the time of diagnosis had a higher risk for calcinosis. Calcinosis was also more common in children with diagnostic delay and chronic persistent disease course. None of these parameters remained independent risk factors for calcinosis in multivariate logistic regression analysis. CONCLUSION: The rate of mortality has decreased dramatically over decades in JDM, but the rate of calcinosis has not changed proportionately. Long duration of active, untreated disease is accepted as the main risk factor for calcinosis. We have seen that calcinosis was more common in children having myalgia, livedo racemosa, skin hypopigmentation, lower ALT levels, and higher physician visual analog scores at the time of diagnosis. [ABSTRACT FROM AUTHOR]

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 (푝 < .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. [ABSTRACT FROM AUTHOR]