34 research outputs found
The Libyan Arabic version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)
No full textThe 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 Libyan Arabic 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 100 JIA patients (22.0% systemic, 26.0% oligoarticular, 25.0% RF negative polyarthritis, and 27.0% other categories) and 100 healthy children, were enrolled in a paediatric rheumatology centre. The JAMAR components discriminated well healthy subjects from JIA patients. Notably, there is no significant difference between the healthy subjects and their affected peers in the school-related problems variable. All JAMAR components revealed satisfactory psychometric performances. In conclusion, the Libyan Arabic 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
Anakinra treatment for systemic onset juvenile idiopathic arthritis in Libyan Children
No full textSystemic onset juvenile idiopathic arthritis (SoJIA) is a rare inflammatory disorder. It is the severest form of juvenile idiopathic arthritis, and complications occur most commonly in this type. Non-responsiveness to standard therapy with corticosteroids and disease-modifying antirheumatic drugs is not uncommon. Interleukin-1 beta (Il-1β) has been shown to be a main contributor to the pathogenesis of SoJIA. Anakinra, a recombinant Il-1β receptor antagonist, was shown to be effective in small cohorts of therapy-resistant adult and pediatric still's patients. This study aimed to evaluate the real-world efficacy, steroid-sparing effect, and safety profile of anakinra in patients with SoJIA at a tertiary care center in Libya. A retrospective case series was conducted on patients with SoJIA treated with anakinra at the Tripoli Children's Hospital between 2010 and 2017. Data on demographic characteristics, disease activity, corticosteroid dosage, concomitant medications, and adverse events were collected at baseline and at 1-, 3-, 6-, and 12-month post-treatment. 13 patients were treated with anakinra with a mean age of 9.4±4.6 years at anakinra initiation and a female-to-male ratio of 2: 1. All patients were on corticosteroids and 92.3% on methotrexate at treatment initiation. The proportion of patients achieving inactive disease (Jadas 10=0) increased over time. A marked steroid-sparing effect was observed: the number of patients requiring high-dose steroids (>0.5 mg/kg/day) decreased from 100% at baseline to 7.6% at 12 months, and 53.8% successfully discontinued corticosteroids entirely. All patients experienced injection site reaction, and macrophage activation syndrome occurred in 15.4% as a side effect after treatment initiation. But no severe infections or fatalities occurred. Reasons for discontinuation included remission (46.1%), drug unavailability (23.0%), inefficacy (15.3%), and side effects (15.3%). Anakinra demonstrated significant efficacy in inducing rapid disease control and reducing corticosteroid dependence in patients with refractory SoJIA, with a manageable safety profile
Additional stresses on buildings induced by vibration effects
No full textAbstractConstruction activities, blasting and traffic are the main sources of ground vibrations that may have a detrimental effect on buildings.The main objective of this research is to study the relationship between additional stresses on building induced by vibrations, vibration nature and building dynamic characteristics.Five numerical models were subjected to base excitation to represent different vibration sources and wave forms. These waves were scaled to the same peak acceleration.Experimental dynamic tests were carried out using five physical models with different characteristics. These models were subjected to different types of vibration. The vibration characteristics were identified and the models top acceleration responses were measured. Moreover, the models maximum stresses were measured due to each vibration load.The relationships between stress increasing, fundamental period and base excitation type were plotted; also the relationship between pseudo acceleration response at the top of the building and the frequency ratio between the model fundamental frequency and the base excitation predominant frequency was also plotted. These relations were analyzed and concluded to tangible results
REFLEXIONS ENTORN DEL SISTEMA ELECTORAL DEL PARLAMENT DE CATALUNYA: EFECTES, PROPOSTES I LÍMITS
No full textThis article examines the particulareffects that the electoral systemapplicable to Catalonia have hashad on the last two elections (in1999 and in 2003). These effects,quite anomalous for a proportionalsystem, meant that the mostvotedpolitical party did not obtain,in turn, the largest numberof Parliamentaing seats. Moreover,these anomalous effects areone of the issues that have openedthe recent political debatefor establishing a new electoralsystem. From this starting point,the author firstly analyzes theconcrete electoral and politicalconsequences derived from thedifferent elements of the electoralsystem with one objective: to pinpointthose elements that couldbe subject to reform. Secondlyand in the light of this previousanalysis, the author presents anddiscusses the different electoralsystem proposals supported bythe main Catalan political parties.He finally provides some generalconclusions about a possible newelectoral system
Tocilizumab effectiveness in paediatric non-infectious uveitis: data from the International AIDA Network Registries on ocular inflammatory disorders
No full textTo describe tocilizumab (TCZ) effectiveness in 15 children with refractory non-infectious uveitis. Reported outcomes are the number of relapses before and after treatment, steroid-sparing effect and drug retention rate. Macular oedema, fluorangiographic findings and ocular complications are also reported. The mean number of ocular relapses significantly decreased from 314 per 100 eyes/year to 106 per 100 eyes/year (p=0.016). A significant steroid-sparing effect was detected (p=0.037). TCZ drug survival was 77.4% at 6 months, followed by 61.9% at 12, 24 and 36 months of follow-up. Macular oedema and retinal vasculitis resolved in all affected eyes
Development of the paediatric society of the African league against rheumatism (PAFLAR) JIA registry and clinical profile of JIA in Africa from the PAFLAR JIA registry
No full textImaging findings in patients with axial spondyloarthritis presenting with recurrent fever attacks: data from the international AIDA network spondyloarthritis registry
No full textSpondyloarthritis (SpA) is a group of immuno-mediated diseases likely caused by a complex interaction between autoimmune and autoinflammatory immunological mechanisms, where a febrile clinical presentation can be an early manifestation. This retrospective study aims to assess the prevalence of inflammatory involvement of the sacroiliac joints (SIJs), pelvis, and lumbosacral spine in patients with axial-SpA and recurrent febrile presentation. MR examinations of 57 patients fulfilling the axial-SpA according to ASAS criteria and presenting with febrile symptoms were evaluated, compared to 30 patients with axial-SpA and no febrile symptoms. 20/57 patients in the axial-SpA group with recurrent fevers underwent a US examination of the SIJs. Structural damage and inflammatory alterations of the SIJs were highly prevalent in both groups. In patients with febrile syndrome, bone marrow edema (78.9%) and erosions (85.9%) were the most prevalent findings in the SIJs; SPARCC score was significantly higher in patients with typical axial-SpA onset (BME: 20.1 ± 12.28 vs. 6.15 ± 3.21; erosions: 22.16 ± 8.13 vs. 6.60 ± 3.08; p = 0.01). Among pelvic enthesitis, enthesitis of the pubic symphysis showed a significant difference in prevalence (p < 0.001). Significant differences were found for the prevalence of vertebral body corner sclerosis (p = 0.01), zygapophyseal capsulitis (p = 0.005), and interspinous enthesitis (p = 0.006). No significant correlation was found between ultrasound findings and MR inflammatory changes (p > 0.05). SIJs and spinal inflammatory alterations and pelvis enthesitis were highly prevalent in axial-SpA patients with and without recurrent fever. Enthesitis of the pubic symphysis, vertebral body corner sclerosis, zygapophyseal capsulitis, and interspinous enthesitis showed a significant difference in frequency between axial-SpA patients with and without fever attacks
Determinants of Discordance Between Criteria for Inactive Disease and Low Disease Activity in Juvenile Idiopathic Arthritis
No full textObjective
To assess concordance among criteria for inactive disease (ID) and low disease activity (LDA) in juvenile idiopathic arthritis (JIA) and to seek factors driving discordance.
Methods
The frequency of fulfillment of existing criteria was evaluated in information on 10,186 patients extracted from 3 cross-sectional data sets. Patients were divided up according to the functional phenotypes of oligoarthritis and polyarthritis. Concordance between criteria was examined using weighted Venn diagrams. The role of each individual component in explaining discordance between criteria was assessed by calculating the absolute number and percentage of instances in which the component was responsible for discrepancy between definitions.
Results
Criteria for ID were met by 28.6–41.1% of patients with oligoarthritis and by 24.0–33.4% of patients with polyarthritis. Criteria for LDA were met by 44.8–62.4% of patients with oligoarthritis and by 44.6–50.4% of patients with polyarthritis. There was a 57.9–62.3% overlap between criteria for ID and a 67.9–85% overlap between criteria for LDA. Parent and physician global assessments and acute-phase reactants were responsible for the majority of instances of discordance among criteria for ID (8.7–15.5%, 10.0–12.3%, and 10.8–17.3%, respectively).
Conclusion
We found fair concordance between criteria for ID and LDA in JIA, with the main drivers of discordance for ID being physician and parent global assessments and acute-phase reactants. This observation highlights the need for further studies aimed to evaluate the impact of subjective physician and parent perception of disease remission and of laboratory measures of inflammatory activity on the definition of ID
Worldwide evaluation of Clinical Practice Strategies (CliPS) for lung involvement in Still's disease within the JIR-CliPS network: a COST action
No full textIncidence and Predictors of Ocular Complications in Pediatric-Onset Uveitis: Data from the AIDA Network Uveitis Registry
No full textIntroduction This study aims to describe complications of pediatric-onset uveitis and their predictors among baseline and treatment-related factors. Methods This registry-based observational study included patients with noninfectious uveitis with disease onset < 18 years. Results A total of 309 patients were enrolled (535 eyes). Uveitis was anterior in 290 eyes (54.2%), panuveitis in 121 (22.6%), intermediate in 88 (16.4%), and posterior in 24 (4.5%). Over a median follow-up of 49.0 months (interquartile range [IQR] 101.0), 137 children (44.3%) developed >= 1 complication (14.4 per 100 patient-years).Idiopathic uveitis (p < 0.001), longer topical glucocorticoid (GC) monotherapy (p < 0.001) and longer delay of immunosuppressive therapy (IST) (p = 0.03) were associated with a higher frequency of complications. In multivariate analysis, anterior uveitis was protective against complications (odds ratio [OR] 0.10, 95% confidence interval [CI] - 4.1 to - 1.6, p < 0.001), whereas a chronic course of uveitis significantly increased the risk (OR 6.13, 95% CI 1.0-2.6, p < 0.001). Older age at onset was protective against cataract (OR 0.91, 95% CI - 0.2 to - 0.02, p = 0.020) and band keratopathy (OR 0.8, 95% CI - 0.4 to - 0.1, p = 0.003).Final best-corrected visual acuity (BCVA) (Snellen decimals) was inversely correlated with the duration of topical GC monotherapy (rho = - 0.23; p = 0.001). In multivariate analysis, panuveitis was linked to a 0.142 decimal reduction (95% CI - 0.219 to - 0.066, p < 0.001), and cataract to a 0.295 reduction (95% CI - 0.372 to - 0.217, p < 0.001) in the final BCVA. Conclusions Children with chronic, idiopathic, early-onset, and non-anterior uveitis are at greatest risk for complications. Structured screening for these children, along with early initiation of systemic IST, is essential to prevent visual impairment
