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Lipponen, L., M. Rahikainen, K. Hakkarainen, and T. Palonen. "Effective Participation and Discourse through a Computer Network: Investigating Elementary Students' Computer Supported Interaction." Journal of Educational Computing Research 27, no. 4 (2002): 355–84. http://dx.doi.org/10.2190/mgtw-qg1e-g66e-f3ud.
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In this study we analyzed and described how students and teacher of one Finnish elementary class participated in and communicated through CSILE (Computer Supported Intentional Learning Environment) during the years 1997–1998. To this end, we examined the density of the interaction within class, students' participation rates, students' position in the CSILE mediated network of interaction, and the quality of their discourse. The data consisted of CSILE log files and students' written productions from the CSILE database. Social network analysis and qualitative content analysis were used to analyze the data. The findings showed that the density of interaction among participants was rather high, and all the students used CSILE to some extent. There were, however, substantial differences in the students' participation rates. The results also indicated that one student occupied a central position, and two students an isolated position in the CSILE mediated interaction. The study further revealed that the CSILE mediated discussion was composed of number of short discussion threads. The culture of CSILE mediated discourse and collaboration can be defined as follows; on topic, neutral, and providing information to others' comments or questions.
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Oshima, Jun. "Differences in Knowledge-Building between Two Types of Networked Learning Environments: An Information-Flow Analysis." Journal of Educational Computing Research 19, no. 3 (1998): 329–51. http://dx.doi.org/10.2190/yllx-m9cw-15x9-bjj9.
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The main purpose of the present study was to examine how elementary school students improve their scientific discourse on a computer-networked database environment called “Computer-Supported Intentional Learning Environments (CSILE).” Students in two, five to six grade combined classrooms taught by the same teacher engaged in their computer-mediated collaborative learning for five weeks by utilizing different system configurations: single-note based (S-CSILE) vs. discussion-note based (D-CSILE). Tracking files automatically recorded by the system were used for an analysis of students' learning activities. Results showed that system affordance specially designed for joint written discourse in D-CSILE significantly facilitated students' joint knowledge-transformation activities as well as maintained each individual's activity to pursue her own agenda. Importance of such a system affordance was discussed from the perspective of distributed cognition.
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Ryser, Gail R., James E. Beeler, and Carol M. McKenzie. "Effects of a Computer-Supported Intentional Learning Environment (CSILE) on Students' Self-Concept, Self-Regulatory Behavior, and Critical Thinking Ability." Journal of Educational Computing Research 13, no. 4 (1995): 375–85. http://dx.doi.org/10.2190/xlgb-pxec-bvxg-grkn.
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Differences in eighth graders' self-concept, self-regulatory behavior, and critical thinking ability between two groups—one group using a software package called CSILE (Computer-Supported Intentional Learning Environment) in a constructivist learning environment and one control group—were investigated. Data were gathered over a one-year period using a quasi-experimental pre-posttest nonequivalent control group design. Results indicated that the CSILE group had a higher level of self-regard, improved ability to regulate their behavior and an increased ability to make credible judgments about someone else's assertions than did the control group ( p < 0.05). The findings are consistent with Scardamalia's assertion that CSILE emphasizes critical thinking skills [1]. Additionally, students in the CSILE group were in a student-directed learning environment which improved their self-concept and may have transferred to their ability to manage their behavior.
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Scardamalia, Marlene, Carl Bereiter, Robert S. McLean, Jonathan Swallow, and Earl Woodruff. "Computer-Supported Intentional Learning Environments." Journal of Educational Computing Research 5, no. 1 (1989): 51–68. http://dx.doi.org/10.2190/cyxd-6xg4-ufn5-yfb0.
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CSILE, which stands for Computer-Supported Intentional Learning Environments, is an educational knowledge media system. CSILE allows information in several media (text, drawings, graphs, timelines, etc.) to be entered into a common database where it is available to be retrieved, linked, commented on, rated, and so forth. The environments and operations of CSILE are designed to support students in more purposeful and mature, or intentional, processing of information. In this article eleven principles, based on recent cognitive research, are suggested for designing computer environments that support intentional learning. These principles include making knowledge-construction activities overt, maintaining attention to learning goals as opposed to other goals of an activity, providing process-relevant feedback, and giving students responsibility for contributing to each other's learning. Applications of these principles in CSILE are described, as well as observations from the first year of school try-out.
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Apiola, Mikko, Matti Lattu, and Tomi A. Pasanen. "Creativity-Supporting Learning Environment---CSLE." ACM Transactions on Computing Education 12, no. 3 (2012): 1–25. http://dx.doi.org/10.1145/2275597.2275600.
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Kang, Dong-Hun, Yang-Ha Hwang, Yong-Sun Kim, Geum Ye Bae, and Seung Jae Lee. "Cognitive Outcome and Clinically Silent Thromboembolic Events After Coiling of Asymptomatic Unruptured Intracranial Aneurysms." Neurosurgery 72, no. 4 (2012): 638–45. http://dx.doi.org/10.1227/neu.0b013e3182846f74.
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Abstract BACKGROUND: Thromboembolic events are the most common complication after coiling of unruptured intracranial aneurysms (UIAs). However, it remains unclear whether these clinically silent ischemic lesions (CSILs) have any clinical significance. OBJECTIVE: To evaluate cognitive outcome after coil embolization of asymptomatic UIAs and its relationship with CSILs after the procedure. METHODS: We prospectively enrolled 40 UIA patients who showed no new focal neurological deficit after coil embolization. CSILs were assessed with diffusion-weighted imaging (DWI) within 1 day after the procedure. A battery of neuropsychological tests was performed 3 times: preoperatively and postoperatively at 1 and 4 weeks after coil embolization. RESULTS: The incidence of cognitive impairment after coiling in patients with UIAs was 44% (17 of 39) at 1 week and 19% (7 of 37) at 4 weeks after coil embolization. DWI within 1 day after coil embolization revealed that 60% of patients (24 of 40) showed CSILs. However, no significant difference was found in any mean cognitive scores or in the number of cognitively impaired variables between patients with and without CSILs at weeks 1 and 4. Additional correlation analysis revealed no correlations between the number of CSILs on DWI and the cognitive sum z score at both 1 and 4 weeks. CONCLUSION: Exhaustive neuropsychological evaluation of UIA patients who underwent coil embolization demonstrated recovery or improvements from baseline cognitive function after 4 weeks, although some patients still showed cognitive deficits at 4 weeks after the procedure. However, we found no statistically significant relationship between the presence and number of CSILs on DWI and cognitive changes after the procedure.
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Akan, Ekemini, Shanmuganathan Chandrakasan, Kelly Rouster-Stevens, et al. "2114 Severity of childhood-onset systemic lupus erythematosus: Impact of preceding and co-existing autoimmune cytopenias (protocol)." Journal of Clinical and Translational Science 2, S1 (2018): 27. http://dx.doi.org/10.1017/cts.2018.120.
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OBJECTIVES/SPECIFIC AIMS: The goals of our study are: (1) To test the hypothesis that the presence of any autoimmune cytopenia (ITP, AIHA, or ES) at time of cSLE diagnosis is associated with decreased risk of developing LN. (1b) To test the hypothesis that there is a lower risk of LN in patients with cSLE and any co-existing autoimmune cytopenia (ITP, AIHA, or ES) who had treatment with immunomodulatory or immunosuppressive therapy (intravenous immunoglobulin, corticosteroids, rituximab, or cyclophosphamide) before diagnosis of cSLE. (2) To test the hypothesis that in patients with cSLE who develop LN, the presence of any co-existing autoimmune cytopenia (ITP, AIHA, or ES) at time of cSLE diagnosis is associated with less severe LN. (3) To test the hypothesis that at the time of cSLE diagnosis, there is a lower incidence of double-stranded DNA (dsDNA) and a higher incidence of ribonucleoprotein autoantibodies in those with co-existing autoimmune cytopenias (ITP, AIHA, or ES). METHODS/STUDY POPULATION: This is a retrospective study of a large cohort of patients from the Emory Children’s Center, Children’s Healthcare of Atlanta (CHOA) satellite clinics and pediatric rheumatology inpatient services at any of the 3 CHOA hospitals (Egleston, Scottish Rite, and Hughes Spalding) with ICD 9 or ICD 10 codes corresponding to a diagnosis of SLE between January 1, 2000 and January 31, 2015. We will include patients diagnosed at age 2–16 years who meet at least 4 of the 11 American College of Rheumatology (ACR) classification criteria for SLE. We will consider these patients as having cSLE. We will exclude patients with less than 2 years of follow-up data and patients with a pre-existing diagnosis of cSLE who transferred care to our Emory/CHOA center. We will define time of diagnosis as time from initial evaluation for cSLE by a pediatric rheumatologist up to 28 days post cSLE diagnosis. We will define co-existing autoimmune cytopenia as preceding diagnosis of a primary autoimmune cytopenia or the presence of an autoimmune cytopenia at the time of initial evaluation for cSLE and up to 28 days post cSLE diagnosis. We will define AIHA as hemoglobin ≤10 g/dL with positive direct Coombs and/or reticulocytosis. We will define ITP as thrombocytopenia <100,000/mm3 and Evans syndrome as concurrent or sequential AIHA and ITP. We will define lupus nephritis (LN) as the presence of urine protein to creatinine ratio>0.5 in a patient with cSLE and/or biopsy demonstrating LN. IRB approval of the study protocol with waiver of informed consent has been obtained from the CHOA IRB. RESULTS/ANTICIPATED RESULTS: We have approximately 40 newly diagnosed cSLE patients annually; therefore, a study population of 400 patients with cSLE is possible. Therefore, assuming 50% of cSLE patients without autoimmune cytopenias have LN and 22% of cSLE patients with autoimmune cytopenias have LN, at an alpha of 0.05, we will have > 80% power to detect significant differences. We expect to show phenotypic differences in patients with co-existing autoimmune cytopenia and cSLE from other newly diagnosed cSLE patients. We expect that the presence of a co-existing autoimmune cytopenia and cSLE is associated with decreased risk of developing LN. We expect that there will be a decreased prevalence of LN in cSLE patients pretreated with immunosuppression further highlighting that earlier indicators of LN risk and early interventions are necessary. We expect to find decreased severity of LN in patients with a co-existing autoimmune cytopenia at time of cSLE diagnosis. DISCUSSION/SIGNIFICANCE OF IMPACT: Our study will be conducted on one of the largest single-center cohorts of cSLE patients. We will determine whether pediatric patients with SLE and autoimmune cytopenias have a distinct clinical or serological phenotype and less severe disease. Our results will be significant in developing hypothesis for further retrospective or prospective multi-center or large database and immunological studies to understand the relationship of each individual autoimmune cytopenia to cSLE. It will provide the necessary background for further clinical and immunological studies to identify predictive biomarkers of cSLE severity.
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Ogbu, Ekemini A., Shanmuganathan Chandrakasan, Kelly Rouster-Stevens, et al. "Impact of autoimmune cytopenias on severity of childhood-onset systemic lupus erythematosus: A single-center retrospective cohort study." Lupus 30, no. 1 (2020): 109–17. http://dx.doi.org/10.1177/0961203320969806.
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Objective To assess whether children with autoimmune cytopenias prior to or at diagnosis of systemic lupus erythematosus (cSLE), differ phenotypically from other cSLE patients; and have a lower risk and severity of lupus nephritis (LN) as observed in prior adult studies. To assess the effect of prior immune therapy for autoimmune cytopenias on 2-year risk of LN. Methods This was a retrospective cohort study of incident cSLE cases. We included patients aged less than 17 years at diagnosis. We excluded patients with LN at cSLE diagnosis. Our follow-up period was 2 years. We defined autoimmune cytopenias as either autoimmune hemolytic anemia, immune thrombocytopenia or Evan’s syndrome. Results Forty-three (33%) of the 130 patients had autoimmune cytopenias before or at cSLE diagnosis. Those with autoimmune cytopenias had significantly more neuropsychiatric symptoms and higher mean ESR but less arthritis, malar rash and myositis versus those without autoimmune cytopenias. They had lower 2-year incidence proportion of LN compared to other cSLE patients (7% vs 15%). Of the 16 patients who developed LN, those with autoimmune cytopenias had mostly class V (2 of 3 patients) versus mostly class III and IV in those without autoimmune cytopenias (6 of 12 patients). None of the 13 patients pre-treated for autoimmune cytopenias developed LN. Conclusion Patients with autoimmune cytopenias before or at cSLE diagnosis have intriguing differences from other cSLE patients. They may represent a unique sub-type of cSLE patients and should be further explored.
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Joo, Young Bin, So-Yeon Park, Soyoung Won, and Sang-Cheol Bae. "Differences in Clinical Features and Mortality between Childhood-onset and Adult-onset Systemic Lupus Erythematosus: A Prospective Single-center Study." Journal of Rheumatology 43, no. 8 (2016): 1490–97. http://dx.doi.org/10.3899/jrheum.151129.
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Objective.To compare clinical features and mortality between childhood-onset systemic lupus erythematosus (cSLE) and adult-onset SLE (aSLE) in a prospective single-center cohort.Methods.A total of 1112 patients with SLE (133 cSLE and 979 aSLE) were enrolled and followed from 1998 to 2012. The 2 groups were compared regarding American College of Rheumatology (ACR) classification criteria for SLE, autoantibodies, disease activity measured by the Adjusted Mean SLE Disease Activity Index (AMS), damage measured by the Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI), and medication. The standardized mortality ratio (SMR) was calculated. Predictors of mortality in SLE were evaluated using Cox proportional hazard models.Results.After a mean followup of 7.6 years, patients with cSLE had a higher number of cumulative ACR criteria and a higher AMS (p < 0.001 each), but there was no difference in SDI (p = 0.797). Immunosuppressants were used more frequently by patients with cSLE (p < 0.001). The SMR of cSLE was 18.8 (95% CI 8.6–35.6), significantly higher than that of aSLE (2.9, 95% CI 2.1–3.9). We found cSLE to be an independent predictor of mortality (HR 3.6, p = 0.008). Moreover, presence of hemolytic anemia (7.2, p = 0.034) and antiphospholipid antibody (aPL; 3.8, p = 0.041) increased the magnitude of risk of early mortality more in the patients with cSLE than in those with aSLE.Conclusion.The clinical course of cSLE as measured by number of clinical manifestations and disease activity is worse than that of aSLE. Also, cSLE patients with hemolytic anemia and aPL are at greater risk of death than patients with aSLE who have those features.
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Groot, Noortje, Nienke de Graeff, Tadej Avcin, et al. "European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative." Annals of the Rheumatic Diseases 76, no. 11 (2017): 1788–96. http://dx.doi.org/10.1136/annrheumdis-2016-210960.
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Childhood-onset systemic lupus erythematosus (cSLE) is a rare, multisystem and potentially life-threatening autoimmune disorder with significant associated morbidity. Evidence-based guidelines are sparse and management is often based on clinical expertise. SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimise and disseminate management regimens for children and young adults with rheumatic diseases like cSLE. Here, we provide evidence-based recommendations for diagnosis and treatment of cSLE. In view of extent and complexity of cSLE and its various manifestations, recommendations for lupus nephritis and antiphospholipid syndrome will be published separately. Recommendations were generated using the EULAR (European League Against Rheumatism) standard operating procedure. An expert committee consisting of paediatric rheumatologists and representation of paediatric nephrology from across Europe discussed evidence-based recommendations during two consensus meetings. Recommendations were accepted if >80% agreement was reached. A total of 25 recommendations regarding key approaches to diagnosis and treatment of cSLE were made. The recommendations include 11 on diagnosis, 9 on disease monitoring and 5 on general treatment. Topics included: appropriate use of SLE classification criteria, disease activity and damage indices; adequate assessment of autoantibody profiles; secondary macrophage activation syndrome; use of hydroxychloroquine and corticosteroid-sparing regimens; and the importance of addressing poor adherence. Ten recommendations were accepted regarding general diagnostic strategies and treatment indications of neuropsychiatric cSLE. The SHARE recommendations for cSLE and neuropsychiatric manifestations of cSLE have been formulated by an evidence-based consensus process to support uniform, high-quality standards of care for children with cSLE.
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Kim, Hyein, Deborah M. Levy, Earl D. Silverman, et al. "A comparison between childhood and adult onset systemic lupus erythematosus adjusted for ethnicity from the 1000 Canadian Faces of Lupus Cohort." Rheumatology 58, no. 8 (2019): 1393–99. http://dx.doi.org/10.1093/rheumatology/kez006.
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Abstract Objective Childhood-onset SLE (cSLE) manifests differently than adult-onset SLE (aSLE). This study determined whether ethnic differences contribute to the differences in clinical presentation between the two groups. Methods This cross-sectional study used data from a multi-centred registry from eight adult and four paediatric Canadian centres gathered at study entry. We compared the frequency of clinical manifestations and autoantibodies between aSLE and cSLE. For those with a significant difference, a multivariable logistic regression was performed, adjusting for ethnicity, SLE onset (cSLE vs aSLE), disease duration and centre. Disease activity and damage between aSLE and cSLE were compared after stratifying by disease duration. Results Of 552 aSLE subjects, 502 (90.9%) were female and 381 (69.0%) were Caucasian. Mean age at diagnosis was 37.0 ± 13.6 years and disease duration 10.9 ± 9.6 years. Of 276 cSLE subjects, 231 (83.7%) were female and 101 (36.6%) were Caucasian. Mean age at diagnosis was 12.7 ± 3.3 years and disease duration 5.6 ± 8.2 years. In multivariable regression analysis, aSLE was associated with decreased odds of having a neurologic disorder (odds ratio = 0.49) and increased odds of having aCL antibodies (odds ratio = 1.85). Disease activity and damage accrual scores were higher in aSLE than cSLE within the same disease duration strata, although the differences were not clinically significant. Ethnicity was not associated with any differences in clinical manifestations or autoantibody frequency between aSLE and cSLE. Conclusion Although a crude comparison of aSLE and cSLE yielded several differences in clinical symptoms and autoantibodies, this difference was not attributable to ethnic differences between aSLE and cSLE.
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Sinicato, Nailú Angélica, Mariana Postal, Fernando Augusto Peres, et al. "Obesity and Cytokines in Childhood-Onset Systemic Lupus Erythematosus." Journal of Immunology Research 2014 (2014): 1–6. http://dx.doi.org/10.1155/2014/162047.
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Background. In systemic lupus erythematosus (SLE), atherosclerosis is attributed to traditional and lupus related risk factors, including metabolic syndrome (MetS), obesity, and inflammation.Objective. To evaluate the association between obesity, measures of body fat content, serum tumor necrosis factor alpha (TNF-α), and interleukin (IL)-6 and -10 levels in childhood-onset SLE (cSLE).Methods. We screened consecutive cSLE patients followed up in the Pediatric Rheumatology Outpatient Clinic of the State University of Campinas. cSLE patients were assessed for disease and damage. Obesity was definite as body mass index (BMI) ≥30 kg/m2. Serum TNF-α, IL-6, and IL-10 levels were measured by ELISA. Dual-energy X-ray absorptiometry was used to determine total fat mass, lean mass, and percent of body fat.Results. We included 52 cSLE patients and 52 controls. cSLE patients had higher serum TNF-α (P=0.004), IL-6(P=0.002), and IL-10(P<0.001)levels compared to controls. We observed higher serum TNF-α (P=0.036)levels in cSLE patients with obesity. An association between serum TNF-αlevels and body fat percent(P=0.046)and total fat mass on trunk region(P=0.035)was observed.Conclusion. Serum TNF-αlevels were associated with obesity and body fat content in cSLE. Our finding suggests that obesity may contribute to the increase of serum TNF-αlevels in cSLE.
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Hersh, A. O., S. M. Case, and M. B. Son. "Predictors of disability in a childhood-onset systemic lupus erythematosus cohort: results from the CARRA Legacy Registry." Lupus 27, no. 3 (2017): 494–500. http://dx.doi.org/10.1177/0961203317747713.
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Objective Few descriptions of physical disability in childhood-onset SLE (cSLE) exist. We sought to describe disability in a large North American cohort of patients with cSLE and identify predictors of disability. Methods Sociodemographic and clinical data were obtained from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry for patients with cSLE enrolled between May 2010 and October 2014. The Childhood Health Assessment Questionnaire (CHAQ) was used to assess disability and physical functioning. Chi-square tests were used for univariate analyses, and multivariate logistic regression was used to assess predictors of disability. Results We analyzed data for 939 patients with cSLE. The median and mean CHAQ scores were 0 and 0.25, respectively, and 41% of the cohort had at least mild disability. Arthritis and higher pain scores were significantly associated with disability as compared to those without disability ( p < 0.001). In multivariate logistic regression analysis, low annual income, arthritis, and higher pain scores were associated with disability at baseline. Conclusions Disability as measured by baseline CHAQ was fairly common in cSLE patients in the CARRA Legacy Registry, and was associated with low household income, arthritis, and higher pain scores. In addition to optimal disease control, ensuring psychosocial supports and addressing pain may reduce disability in cSLE. Further study is needed of disability in cSLE.
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Brunner, Hermine I., Alberto Martini, Daniel J. Lovell, and Nicolino Ruperto. "Clinical trials in children and adolescents with systemic lupus erythematosus: methodological aspects, regulatory landscape and future opportunities." Annals of the Rheumatic Diseases 78, no. 2 (2018): 162–70. http://dx.doi.org/10.1136/annrheumdis-2018-213198.
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Childhood-onset systemic lupus erythematosus (cSLE) is rare in many regions of the world, including Europe. Access to approved medications for cSLE is currently limited, among others, due to a lack of high-quality evidence from clinical trials. The objectives of the study were to evaluate the current regulatory framework regarding medication approvals, delineate barriers to clinical trial conduct, and strategies to improve access to new medications for cSLE. Relevant methodological and regulatory aspects, epidemiological data, study designs and outcome measures are reviewed, and the results of a survey among Paediatric Rheumatology International Trials Organisation/Pediatric Rheumatology Collaborative Study Group investigators are presented. Laws and regulations in the USA and Europe necessitate that novel medicines are studied in paediatric populations, if similar or the same diseases in adults have been found to benefit from them. Regulatory agencies consider cSLE the paediatric form of SLE in adults. For medicines that have been found safe and effective in adult SLE, paediatric extrapolation strategies can limit the number and complexity of studies needed to support the labelling of these medicines for use in cSLE. In this setting, specialised research networks, validated outcome measures, stakeholder input, study designs as well as statistical methods successfully used in other uncommon diseases will help improve study efficiency in an effort to enhance the speed with which new drugs for cSLE can be studied. Open-label pharmacokinetic-pharmacodynamic studies are preferred by paediatric rheumatologists over double-blind parallel designs for cSLE trials. Appropriate infrastructure, outcome measures and sufficient numbers of patients are available for the testing of new medicines for children with cSLE.
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BRUNNER, HERMINE I., GLORIA C. HIGGINS, KRISTINA WIERS, et al. "Health-related Quality of Life and Its Relationship to Patient Disease Course in Childhood-onset Systemic Lupus Erythematosus." Journal of Rheumatology 36, no. 7 (2009): 1536–45. http://dx.doi.org/10.3899/jrheum.081164.
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Objective.To (1) estimate the health-related quality of life (HRQOL) of children with childhood-onset systemic lupus erythematosus (cSLE) and compare it to that of normative cohorts; (2) assess the relationship of HRQOL with cSLE disease activity and damage; and (3) determine the effects of changes of disease activity on HRQOL.Methods.Patients with cSLE (n = 98) followed every 3 months completed HRQOL measures, the Pediatric Quality of Life Inventory Generic Core scale (PedsQL-GC), the Rheumatology Module (PedsQL-RM), and the Child Health Questionnaire (CHQ). The British Isles Lupus Activity Group Index (BILAG) was used to measure organ-system-specific disease activity. Physicians rated the course of cSLE between visits.Results.At baseline, mean (standard deviation, SD) score [parent report] of the PedsQL-GC and the PedsQL-RM was 75 (17) and 79 (14), respectively; the mean (SD) of the CHQ physical summary score (CHQ-PHS) was 49 (7) and that of the CHQ psychological summary score was 42 (12). Higher BILAG scores, especially in the general, musculoskeletal, neurological, and vascular, but not the mucocutaneous, renal, cardiovascular, or hematological BILAG domains, were associated with a significantly lower HRQOL. Patients with damage had lower HRQOL than those without damage. All HRQOL measures included were at most modestly responsive to clinically important changes with cSLE.Conclusion.HRQOL with cSLE is significantly lower than that reported in healthy populations. Organ-specific involvement with cSLE has a differential effect on HRQOL. Higher disease activity and damage are associated with significantly lower HRQOL as measured by the PedsQL-RM and the CHQ-PHS, and worsening of cSLE leads to a further decline.
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Rotstein Grein, Ingrid Herta, Natalia Ferreira Pinto, Aline Lobo, et al. "Safety and immunogenicity of the quadrivalent human papillomavirus vaccine in patients with childhood systemic lupus erythematosus: a real-world interventional multi-centre study." Lupus 29, no. 8 (2020): 934–42. http://dx.doi.org/10.1177/0961203320928406.
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Objective This study aimed to assess the safety and immunogenicity of the quadrivalent human papillomavirus (qHPV) vaccination in childhood-onset systemic lupus erythematosus (cSLE) patients. Methods Volunteer cSLE patients aged 9–20 years and healthy controls (HC) were enrolled to receive a two- or three-dose qHPV vaccination schedule from March 2014 to March 2016. Study visits were performed before the first dose, one month after the second and third doses and one year after the first dose. In each study visit, disease activity and adverse events following vaccination were analyzed, and a serum sample was collected for testing antibody concentrations. Participant recruitment was conducted in 15 Brazilian paediatric rheumatology units. Of the 256 cSLE patients included, 210 completed the two- or three-dose schedules; 15 had previously received one dose, and 18 had received two doses of the vaccine. The analysis was based on intention-to-treat so that participants who did not complete the entire study protocol were also included. Results No severe adverse events were related to the vaccination. Disease activity was generally low and remained stable or even improved. The HC presented 100% seropositivity to HPV16 and HPV18, whereas the two- and three-dose cSLE groups presented 93% and 83% versus 97% and 91%, respectively. One year after the first dose, seropositivity of the three-dose cSLE group was 91% to HPV16 and 84% to HPV18. Conclusions HPV vaccination in cSLE patients is safe and immunogenic. Since the seropositivity to HPV16 and HPV18 was higher for the three-dose schedule group, this regimen should be recommended for cSLE patients.
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Hovde, Aldina M., Cheryl AS McFarland, G. Melissa Garcia, et al. "Multi-pronged approach to enhance education of children and adolescents with lupus, caregivers, and healthcare providers in New Jersey: Needs assessment, evaluation, and development of educational materials." Lupus 30, no. 1 (2020): 86–95. http://dx.doi.org/10.1177/0961203320969975.
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Background Childhood Systemic Lupus Erythematosus (cSLE) patients are younger at diagnosis and have a more severe disease course compared to adult onset SLE patients and develop significant complications related to disease and or immunosuppression. Moreover, female and minority populations experience higher rates of cSLE, with African American, Afro-Caribbean, and Hispanic populations being at greatest risk and having poor prognosis Methods The Pediatric Alliance for Lupus initiative addressed the dearth in education and resources in a multi-stage process. First, we conducted a need assessment identifying knowledge gaps among healthcare providers (HCPs), and resources needed to care for cSLE patients and their families. Second, we educated HCPs about the diagnosis and treatment of cSLE by Continuing Medical Education (CME) sessions/webinars (presented here). Third, HCPs participated in a Quality Improvement (QI) program on cSLE approved by the American Board of Pediatrics Maintenance of Certification Part 4. Finally, patients and caregivers were educated through the development of appropriate, culturally and linguistically sensitive cSLE resources. PAL disseminated materials among HCPs and the community to improve the awareness of the availability of these materials. Results According to results from the statewide needs assessment (representative of every county throughout NJ), HCPs face significant challenges in providing care to cSLE patients and their families, in part due to the multi-systemic nature of the autoimmune disease. Conclusion Based on this need, we developed educational sessions, with pre-post comparison data showing a significant increase in knowledge after HCP education. The 15 different materials developed as part of the endeavor is a major contribution to the cSLE community, HCPs and pediatric rheumatologists. Resources are available in multiple formats (PDF and web pages), and are accessible on the National Resource Center on Lupus, the latest web site of the Lupus Foundation of American that houses materials for SLE patients, their families, schools, HCPs, and the community at large. Improving cSLE knowledge will empower the children and adolescents and families by increasing their self-efficacy; and positively impact key health outcomes (transition readiness and HRQOL) that are not optimally addressed with current medical treatment alone.
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Zahr, Noël, Saik Urien, Christian Funck-Brentano, et al. "Evaluation of Hydroxychloroquine Blood Concentrations and Effects in Childhood-Onset Systemic Lupus Erythematosus." Pharmaceuticals 14, no. 3 (2021): 273. http://dx.doi.org/10.3390/ph14030273.
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Background: Hydroxychloroquine (HCQ) is an antimalarial agent given to patients with systemic lupus erythematosus (SLE) as first-line therapy. It alleviates childhood-onset systemic lupus erythematosus cSLE skin and musculoskeletal disease, decreasing disease activity and flares. HCQ concentration–effect relationships in children remains unknown. This study aimed to investigate the pharmacokinetics of HCQ and possible concentration–effect relationships. Methods: HCQ blood concentrations and effects were obtained during clinical follow-up on different occasions. cSLE flares were defined using the SLE Disease Activity Index (SLEDAI); flare was denoted by a SLEDAI score > 6. Blood concentration was measured using high-performance liquid chromatography with fluorometric detection. Statistical analysis was performed using a nonlinear mixed-effect approach with the Monolix software. Results: A total of 168 blood samples were obtained from 55 pediatric patients. HCQ apparent blood clearance (CL/F) was dependent on patients’ bodyweight and platelet count. Patients with active cSLE had a lower mean blood HCQ concentration compared with inactive cSLE patients (536 ± 294 vs. 758 ± 490 ng/mL, p = 5 × 10−6). Among patients with HCQ blood concentration ≥750 ng/mL, 87.6% had inactive cSLE. Moreover, HCQ blood concentration was a significant predictor of disease status. Conclusion: We developed the first HCQ blood concentration–effect relationship for cSLE associated with active or non-active disease status. A prospective randomized study is necessary to confirm these results.
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Holcar, Marija, Aleš Goropevšek, and Tadej Avčin. "Altered Homeostasis of Regulatory T Lymphocytes and Differential Regulation of STAT1/STAT5 in CD4+ T Lymphocytes in Childhood-onset Systemic Lupus Erythematosus." Journal of Rheumatology 47, no. 4 (2019): 557–66. http://dx.doi.org/10.3899/jrheum.181418.
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Objective.Childhood-onset systemic lupus erythematosus (cSLE) is usually a more severe and aggressive disease than adult-onset SLE (aSLE), but cellular and subcellular reasons for these differences are not well understood. The present study analyzed Th subsets, STAT1/STAT5 signaling response, and cytokine profiles of cSLE.Methods.FOXP3+ regulatory (Treg) and effector Th subsets, expression and phosphorylation of STAT1/STAT5 in Th, and cytokine profiles were measured in the peripheral blood of patients with cSLE and healthy controls (HC), using flow cytometry and immunoassay on a biochip.Results.Significant correlation between expression of the activation marker HLA-DR and decreased Th counts, an increase in the percentage of FOXP3+ Th, and a decrease in the activated Treg (aTreg) subset among them were found in cSLE. In contrast to our previous findings in aSLE, no significant differences in percentages and a significant decrease in the numbers of the naive-resting Treg (rTreg) subset compared to HC were found. The percentages of CD25− cells, possibly reflecting interleukin 2 depletion, were significantly increased in cSLE aTreg, but not in the rTreg subset. Consistent with the results of our previous studies in aSLE, increased expression of STAT1, along with significant correlation between decreased Th counts and their increased basal phosphorylation of STAT5, were also found in cSLE.Conclusion.Our results suggest that the key difference in Treg homeostasis between cSLE and aSLE is in the rTreg subset. However, perturbed aTreg homeostasis, increased levels of STAT1 protein, and homeostatic STAT5 signaling appear to be intrinsic characteristics of the disease, present in cSLE and aSLE alike.
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Harry, O., L. E. Crosby, A. W. Smith, et al. "Self-management and adherence in childhood-onset systemic lupus erythematosus: what are we missing?" Lupus 28, no. 5 (2019): 642–50. http://dx.doi.org/10.1177/0961203319839478.
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Objectives The aims of this study are (1) to characterize factors influencing self-management behaviors and quality of life in adolescent and young adult (AYA) patients with childhood-onset systemic lupus erythematosus (cSLE) and (2) to identify barriers and facilitators of treatment adherence via focus groups. Methods AYAs with cSLE ages 12–24 years and primary caregivers of the adolescents participated in this study. Recruitment occurred during pediatric rheumatology clinic visits at a Midwestern children's hospital or the hospital's cSLE active clinic registry. Information about disease severity was obtained from patient health records. Pain and fatigue questionnaires were administered. Descriptive statistics were used to analyze data. Results Thirty-one AYA patients and caregivers participated in six focus groups. Ten major themes emerged from sessions; four were expressed both by the AYA and caregiver groups: knowledge deficits about cSLE, symptoms limiting daily function, specifically mood and cognition/learning, barriers and facilitators of adherence, and worry about the future. Themes unique to AYA participants included symptoms limiting daily functioning—pain/fatigue, self-care and management, impact on personal relationships, and health care provider communication/relationship. For caregiver groups unique themes included need for school advocacy, disruption of family schedule, and sense of normalcy for their adolescent. Conclusion AYAs with cSLE face a lifelong disease characterized by pervasive pain, fatigue, organ damage, isolation—social and/or physical—and psycho-socioeducational challenges. This study confirmed that continued psychosocial support, health information education, adherence interventions, and personalized treatment plans are necessary to increase self-management and autonomy in AYAs with cSLE.
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Kim, Donghyun, Nok Park, Jeong Hwang, et al. "Camellia sinensis leaf extracts lacking catechins exert depigmentary effects through ERK-dependent, MiTF-mediated tyrosinase downregulationin melan-a cells and a human skin equivalent." Archives of Biological Sciences 71, no. 3 (2019): 483–88. http://dx.doi.org/10.2298/abs190510031k.
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Green tea from Camellia sinensis, a popular beverage worldwide, is also considered a herbal medicine. The bioactive compounds of green tea include polyphenols, polysaccharides, amino acids, and vitamins. Tea polyphenols are composed of various catechins such as epigallocatechin-3-gallate (EGCG), whereas polysaccharides include complex pectic substances and glycoproteins. Unfractionated C. sinensis leaf extracts show various pharmacological effects that are attributable to catechins, which include antimelanogenic properties. Most studies have focused on the biological function of catechins in green tea, and the effects of C. sinensis leaf extracts lacking catechins (CSLE-LC) have received little research attention. We examined the skin-whitening properties of the fraction lacking catechins. The melanin content in melan-a cells was significantly reduced, as has been shown for unfractionated C. sinensis extracts and catechins. We also elucidated the molecular mechanism underlying the antimelanogenic effects of CSLE-LC on skin melanocytes. Our results show that CSLE-LC acts through inhibition of MiTF and subsequent activation of extracellular-signal regulated kinase (ERK) to reduce tyrosinase protein levels. We confirmed the whitening ability of CSLE-LC using a human skin equivalent. Our findings provide the first evidence that CSLE-LC could exert efficient antimelanogenesis activity, and suggest that polysaccharides as well as catechins contribute to the whitening efficacy of C. sinensis leaf extracts.
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Tao, Jessie J., Linda T. Hiraki, Deborah M. Levy, and Earl D. Silverman. "Comparison of Sensitivities of American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria in Childhood-onset Systemic Lupus Erythematosus." Journal of Rheumatology 46, no. 7 (2019): 731–38. http://dx.doi.org/10.3899/jrheum.180337.
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Objective.Currently there are 2 different classification criteria for systemic lupus erythematosus (SLE): American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC). The aim of this study was to compare the sensitivities of ACR and SLICC criteria in childhood-onset SLE (cSLE) using a large, multiethnic cohort.Methods.We conducted a retrospective study of 722 patients diagnosed with cSLE at The Hospital for Sick Children (SickKids). Prospectively collected data from SickKids’ Lupus Database were reviewed/validated against medical records prior to ACR and SLICC scoring based on cumulative symptoms up to the last visit. Sensitivities were compared using McNemar’s test. Descriptive statistics were used to identify SLE features unique to each set of criteria and autoantibodies not included in either.Results.ACR and SLICC sensitivities were as follows: 92.4% and 96.3% overall (p = 0.001); 82.5% and 91.3% (p = 0.01) in those scored ≤ 1 year from diagnosis; 92.7% and 97.9% (p = 0.02) in those scored 2–3 years from diagnosis. Forty-eight of 55 (87.3%) patients who did not meet ACR criteria met SLICC criteria through SLICC-specific criterion or renal biopsy. Twenty of 27 (74.1%) patients who did not meet SLICC criteria met ACR criteria as a result of photosensitivity (73.9%) and ACR lymphopenia criteria (26.1%). Six of 7 patients (85.7%) who were clinically diagnosed with cSLE but did not meet either SLICC or ACR criteria had anti-Ro antibodies.Conclusion.SLICC criteria were significantly more sensitive than ACR criteria in cSLE classification, especially early in the disease course. Because of the extreme rarity of primary Sjögren syndrome in children, one may consider adding anti-Ro antibodies to the classification criteria for cSLE because they are present in ∼40% of patents with cSLE.
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Imam, A. A., H. E. Ibrahim, M. A. A. Farghaly, et al. "Vitamin D receptor gene FokI polymorphism in Egyptian children and adolescents with SLE: A case-control study." Lupus 26, no. 13 (2017): 1426–34. http://dx.doi.org/10.1177/0961203317725588.
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Background Childhood-onset systemic lupus erythematosus (cSLE) is a lifelong autoimmune disorder. The vitamin D receptor (VDR) gene is a potential candidate gene for cSLE susceptibility. In this study, we aimed to investigate the FokI polymorphism in the VDR gene in Egyptian children and adolescents with SLE, to determine whether this polymorphism could be a genetic marker for cSLE susceptibility or disease activity and we also measured the serum level of 25-hydroxyvitamin D [25(OH) D] to assess its relation to such polymorphism. Methods This was a case-control study, which included 300 patients with cSLE and 300 age, sex, and ethnicity-matched healthy controls. All participants were genotyped for the VDR gene FokI (rs2228570) polymorphism by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP), while the serum [25(OH) D] levels were measured by enzyme-linked immunosorbent assay (ELISA). Results The VDR FokI FF genotype and F allele were overrepresented among cSLE patients compared with the controls, [odds ratio (OR) = 2.7; 95% confidence interval (CI): 1.6–4.4 for the FF genotype; p = 0.000; and OR = 1.6; 95% CI: 1.27–2.05 for the F allele; p = 0.000, respectively]. We found a significant association between VDR FokI FF genotype with lupus nephritis (OR: 4.8; 95% CI: 2.2–10.6; p = 0.002); and high disease activity index score ( p = 0.01). Conclusions The FokI polymorphism in the VDR gene may contribute to susceptibility to SLE in Egyptian children and adolescents. Moreover, the FF genotype constituted a risk factor for the development of lupus nephritis and was associated with low serum [25(OH) D] levels as well as higher disease activity index score among studied patients with cSLE.
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Schonenberg-Meinema, Dieneke, Sandy C. Bergkamp, Amara Nassar-Sheikh Rashid, et al. "Nailfold capillary abnormalities in childhood-onset systemic lupus erythematosus: a cross-sectional study compared with healthy controls." Lupus 30, no. 5 (2021): 818–27. http://dx.doi.org/10.1177/0961203321998750.
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Objectives For selection of high-risk systemic lupus erythematosus (SLE) patients it is necessary to obtain indicators of disease severity that predict disease damage. As in systemic sclerosis, nailfold capillary abnormalities could be such a biomarker in SLE. The primary objective of this cross-sectional study is to describe capillary abnormalities in childhood-onset SLE (cSLE) cohort (onset < 18 years) and compare them with matched healthy controls. The secondary objective is to correlate the observed capillary abnormalities with demographical variables in both cohorts and with disease-specific variables in cSLE patients. Methods Healthy controls were matched for ethnic background, age and gender. Videocapillaroscopy was performed in eight fingers with 2-4 images per finger. Quantitative and qualitative assessments of nailfold capillaroscopy images were performed according to the definitions of the EULAR study group on microcirculation in Rheumatic Diseases. Results Both groups (n = 41 cSLE-patients and n = 41 healthy controls) were comparable for ethnic background (p = 0.317). Counted per mm, cSLE-patients showed significantly more ‘giants’ (p = 0.032), ‘abnormal capillary shapes’ (p = 0.003), ‘large capillary hemorrhages’ (p < 0.001) and ‘pericapillary extravasations’ (p < 0.001). Combined ‘abnormal capillary shapes and pericapillary extravasations’ (in the same finger) were detected in 78% (32/41 patients). By qualitative analysis, ‘microangiopathy’ was detected in 68.3% (28/41) and a ‘scleroderma pattern’ in 17.1% (7/41) of the cSLE-patients (without scleroderma symptoms). The difference of percentage positive anti-RNP antibodies in the group with or without a scleroderma pattern was not significant (p = 0.089). The number of ‘abnormal capillary shapes per mm’ was significantly correlated with treatment-naivety. The number of ‘large pathological hemorrhages per mm’ was significantly correlated with SLEDAI score and presence of nephritis. Compared to healthy controls, ‘pericapillary extravasations’ were found in significantly higher numbers per mm (p < 0.001) as well as in percentage of patients (p < 0.001). Conclusions Our observations confirm that giants, abnormal capillary morphology and capillary hemorrhages are also observed in cSLE, as was already known for adults with SLE. Number of capillary hemorrhages in cSLE was significantly correlated with disease activity. A high frequency and total amount of “pericapillary extravasations” was observed in cSLE patients, possibly revealing a new subtype of capillary hemorrhage that might reflect endothelial damage in these pediatric patients.
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Kiss, Adam K., and Daniel Bachrathy. "EXPERIMENTAL VALIDATION OF CUMULATIVE SURFACE LOCATION ERROR FOR TURNING PROCESSES." Acta Polytechnica CTU Proceedings 3 (February 11, 2016): 25–29. http://dx.doi.org/10.14311/app.2016.3.0025.
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The aim of this study is to create a mechanical model which is suitable to investigate the surface quality in turning processes, based on the Cumulative Surface Location Error (CSLE), which describes the series of the consecutive Surface Location Errors (SLE) in roughing operations. In the established model, the investigated CSLE depends on the currently and the previously resulted SLE by means of the variation of the width of cut. The phenomenon of the system can be described as an implicit discrete map. The stationary Surface Location Error and its bifurcations were analysed and flip-type bifurcation was observed for CSLE. Experimental verification of the theoretical results was carried out.
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Lapa, A. T., M. Postal, N. A. Sinicato та ін. "S100β is associated with cognitive impairment in childhood-onset systemic lupus erythematosus patients". Lupus 26, № 5 (2017): 478–83. http://dx.doi.org/10.1177/0961203317691374.
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Objective To investigate serologic S100β protein levels in childhood-onset SLE patients (cSLE) and to elucidate their association with disease activity and neuropsychiatric (NP) manifestations. Methods We included 71 cSLE patients (67 females; median age 18 years; range 9–37 and 53 (47 females; median age of 20 years; range 6–29) age and sex matched healthy controls. Neurological manifestations were analysed according to the American College of Rheumatology (ACR) criteria. Cognitive evaluation was performed in all participants using Wechsler Intelligence Scale for Children (WISC-III) and Wechsler Adult Intelligence Scale (WAIS), according to age, and validated in Portuguese. SLE patients were further assessed for clinical and laboratory SLE manifestations, disease activity (SLE Disease Activity Index (SLEDAI)), damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)) and current drug exposures. Sera S100β protein levels were measured by enzyme-linked immunosorbent assay using commercial kits. Results The median S100β protein level was 116.55 pg/mL (range 1.53–468.50) in cSLE and 54.98 pg/mL (range 0.69–181.00) in healthy controls ( p < 0.001). An association was observed between S100β protein and NP manifestations ( p = 0.03). The S100β protein levels was associated with cognitive impairment in cSLE patients ( p = 0.006). Conclusions S100β protein levels are increased in cSLE with cognitive impairment. S100β may be considered a potential biomarker that underlies central nervous system (CNS) dysfunction, especially cognitive impairment.
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Quilter, Michelle, Linda Hiraki, Andrea M. Knight, et al. "Evaluation of self-report screening measures in the detection of depressive and anxiety disorders among children and adolescents with systemic lupus erythematosus." Lupus 30, no. 8 (2021): 1327–37. http://dx.doi.org/10.1177/09612033211018504.
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Background There are no validated screening measures for depressive or anxiety disorders in childhood Systemic Lupus Erythematosus (cSLE). We investigated cross-sectionally (1) the prevalence of depressive and anxiety disorder in cSLE. (2) the validity of the Centre for Epidemiologic Studies Depression Scale for Children (CES-DC) and the Screen for Childhood Anxiety and Related Disorders (SCARED) measures in identifyingthese disorders. Methods Participants 8-18 years with cSLE/incipient cSLE completed CES-DC, SCARED, and Quality OfMy Life (QOML) measures. Parents completed the SCARED-Parent measure. Diagnosis was by gold-standard psychiatric interview and determined prevalence of psychiatric disorder. Receiver Operating Characteristics Area under the Curve (ROCAUC) evaluated screening measure diagnostic performance. Results Ofseventy-two parent-child dyads, 56 interviews were completed. Mean screen scores were: CES-DC = 15 (range 1-49, SD 12), SCARED-C = 22 (range 2-61, SD 14), SCARED-P = 13 (range 0-36, SD 8). Depressive disorder screen positivity (CES-DC ≥ 15) was 35% (vs. prevalence 5%). Anxiety disorder screen positivity (SCARED ≥ 25) was 39% (vs. prevalence 16%). CES-DC ROCAUC = 0.98 and SCARED-C ROCAUC = 0.7 (cut-points 38 and 32 respectively). Conclusions Diagnostic thresholds for depressive and anxiety disorderscreening measures are high for both CES-DC and SCARED-C in cSLE. Brief focused interview should follow to determine whether psychiatric evaluation is warranted.
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Webber, Declan, Jingjing Cao, Daniela Dominguez, et al. "Association of systemic lupus erythematosus (SLE) genetic susceptibility loci with lupus nephritis in childhood-onset and adult-onset SLE." Rheumatology 59, no. 1 (2019): 90–98. http://dx.doi.org/10.1093/rheumatology/kez220.
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Abstract Objective LN is one of the most common and severe manifestations of SLE. Our aim was to test the association of SLE risk loci with LN risk in childhood-onset SLE (cSLE) and adult-onset SLE (aSLE). Methods Two Toronto-based tertiary care SLE cohorts included cSLE (diagnosed &lt;18 years) and aSLE patients (diagnosed ⩾18 years). Patients met ACR and/or SLICC SLE criteria and were genotyped on the Illumina Multi-Ethnic Global Array or Omni1-Quad arrays. We identified those with and without biopsy-confirmed LN. HLA and non-HLA additive SLE risk-weighted genetic risk scores (GRSs) were tested for association with LN risk in logistic models, stratified by cSLE/aSLE and ancestry. Stratified effect estimates were meta-analysed. Results Of 1237 participants, 572 had cSLE (41% with LN) and 665 had aSLE (30% with LN). Increasing non-HLA GRS was significantly associated with increased LN risk [odds ratio (OR) = 1.26; 95% CI 1.09, 1.46; P = 0.0006], as was increasing HLA GRS in Europeans (OR = 1.55; 95% CI 1.07, 2.25; P = 0.03). There was a trend for stronger associations between both GRSs and LN risk in Europeans with cSLE compared with aSLE. When restricting cases to proliferative LN, the magnitude of these associations increased for both the non-HLA (OR = 1.30; 95% CI 1.10, 1.52; P = 0.002) and HLA GRS (OR = 1.99; 95% CI 1.29, 3.08; P = 0.002). Conclusion We observed an association between known SLE risk loci and LN risk in children and adults with SLE, with the strongest effect observed among Europeans with cSLE. Future studies will include SLE-risk single nucleotide polymorphisms specific to non-European ancestral groups and validate findings in an independent cohort.
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Valões, C. C. M., B. C. Molinari, A. C. G. Pitta, et al. "Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients." Lupus 26, no. 5 (2017): 484–89. http://dx.doi.org/10.1177/0961203316676386.
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Objectives Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody. Methods This was a retrospective multicenter study performed in 10 pediatric rheumatology services of São Paulo state, Brazil. Anti-P antibodies were measured by ELISA in 228 cSLE patients. Results Anti-P antibodies were observed in 61/228 (27%) cSLE patients. Frequencies of cumulative lymphadenopathy (29% vs. 15%, p = 0.014), acute confusional state (13% vs. 5%, p = 0.041), mood disorder (18% vs. 8%, p = 0.041), autoimmune hemolytic anemia (34% vs. 15%, p = 0.001), as well as presence of anti-Sm (67% vs. 40%, p = 0.001), anti-RNP (39% vs. 21%, p = 0.012) and anti-Ro/SSA antibodies (43% vs. 25%, p = 0.016) were significantly higher in cSLE patients with anti-P antibodies compared to those without these autoantibodies. A multiple regression model revealed that anti-P antibodies were associated with autoimmune hemolytic anemia (odds ratio (OR) = 2.758, 95% confidence interval (CI): 1.304–5.833, p = 0.008) and anti-Sm antibody (OR = 2.719, 95% CI: 1.365–5.418, p = 0.004). The SLICC/ACR damage index was comparable in patients with and without anti-P antibodies ( p = 0.780). Conclusions The novel association of anti-P antibodies and autoimmune hemolytic anemia was evidenced in cSLE patients and further studies are necessary to determine if anti-P titers may vary with this hematological manifestation.
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Zhu, Ying, Zhao Wei, Yexin Li, Jingqi Luo, and Bizhou Ge. "Industry-energy system management by a Copula-based stochastic programming approach." E3S Web of Conferences 267 (2021): 01002. http://dx.doi.org/10.1051/e3sconf/202126701002.
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In this study, a Copula-based stochastic industry-energy system management (CSIE) model was developed based on Copula-based stochastic programming and interval linear programming. CSIE model can not only deal with extreme random events in industry-energy system (IES) of resource-dependent cities, but also quantify the risks of industrial energy demand-supply. To prove the practicability, a case study of IES planning in Yulin city was represented. Reasonable solutions of energy production and industrial energy consumption strategy were obtained, which can guarantee that pollutant emission meets the environmental requirements, and the system cost gets the lowest during 2021-2035. Furthermore, CSIE model could be spread to IES management in similar resource-dependent cities.
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Smitherman, Emily A., Bin Huang, Adam Furnier, et al. "Quality of Care in Childhood-onset Systemic Lupus Erythematosus: Report of an Intervention to Improve Cardiovascular and Bone Health Screening." Journal of Rheumatology 47, no. 10 (2019): 1506–13. http://dx.doi.org/10.3899/jrheum.190295.
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ObjectiveInitial benchmarking of childhood-onset systemic lupus erythematosus (cSLE) quality indicators revealed suboptimal performance across multiple centers. Our aim was to improve cardiovascular and bone health screenings at a tertiary treatment center for cSLE. This included annual measurements of vitamin D, lipid profiles, and bone mineral density through dual-energy x-ray absorptiometry (DXA).MethodsQuality improvement methodology was applied to design and implement a standardized previsit planning process to electronically entered and saved orders for needed screenings prior to a scheduled clinic visit. Process outcomes were measured using statistical process control charts. Univariate analyses were completed to assess patient-level factors.ResultsDuring the study, 123 patients with cSLE participated across 619 clinic visits. The percentage of patients with completed screenings improved from 54% to 92% for annual vitamin D, 55% to 84% for annual lipid profiles, and 57% to 78% for DXA, which was sustained for more than 1 year. Providers responded to a majority of abnormal results, and improvement in the average vitamin D level was observed over time. Higher levels of disease activity, damage, number of clinic visits, and screenings completed at baseline were observed in patients with all screenings completed at the end of the intervention.ConclusionImplementation of elements of the chronic illness care model for cSLE management improved performance of cardiovascular and bone health screenings, a step toward preventing longterm morbidity in cSLE. Our study also suggests that more patient interaction with the healthcare system may promote successful completion of health maintenance screenings.
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Davis, A. M., T. B. Graham, Y. Zhu, and M. L. McPheeters. "Depression and medication nonadherence in childhood-onset systemic lupus erythematosus." Lupus 27, no. 9 (2018): 1532–41. http://dx.doi.org/10.1177/0961203318779710.
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Objectives Depression and medication nonadherence are important in managing chronic diseases, but little is known about these factors in childhood-onset systemic lupus erythematosus (cSLE). The objectives of this cross-sectional study were to estimate prevalence of depression and medication nonadherence, describe demographic and disease characteristics associated with depression and medication nonadherence, and evaluate the association between depression and medication nonadherence in cSLE patients. Methods Patients with cSLE ( n = 51) completed validated screening questionnaires to identify depression and medication nonadherence, Patient Health Questionnaire-9 and Medication Adherence Self-Report Inventory, respectively. Demographic and disease characteristics were obtained via chart abstraction, and compared between groups of depression or medication nonadherence status. A multivariable linear regression model adjusting for propensity scores was conducted to evaluate the association between depression and medication nonadherence. Results The prevalence of a positive depression screen was 58.8%, and seven patients reported suicidal ideation (13.7%). The prevalence of self-reported medication nonadherence was 19.7%. No statistically significant differences for demographic and disease characteristics were found between patients with a positive vs. negative depression screen. Patients reporting medication nonadherence were more likely to have longer disease duration (4.8 vs. 2.6 years, p = 0.035). As the severity of depression symptoms increased, the degree of medication nonadherence also increased (beta = –1.89; p = 0.011). Conclusions The prevalence of depression and medication nonadherence is high in cSLE, and these factors have a direct relationship. Interventions that better recognize and treat depression and increase rates of medication adherence are needed to improve outcomes in cSLE.
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AlAhmed, Ohoud, Vidya Sivaraman, Melissa Moore-Clingenpeel, Stacy P. Ardoin, and Sharon Bout-Tabaku. "Autoimmune thyroid diseases, autoimmune hepatitis, celiac disease and type 1 diabetes mellitus in pediatric systemic lupus erythematosus: Results from the CARRA Legacy Registry." Lupus 29, no. 14 (2020): 1926–36. http://dx.doi.org/10.1177/0961203320961469.
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Objective Polyautoimmunity (PA) with systemic lupus erythematosus (SLE) is reported as a poor prognostic factor, but little is known about its effect in childhood-onset SLE (cSLE). We describe PA in cSLE within the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry and evaluate its association to lupus disease outcomes. Methods CARRA Legacy Registry is the largest pediatric rheumatology registry that collected data at enrollment and every 6 months thereafter. We describe the co-occurrence of selected autoimmune disorders (autoimmune thyroid diseases, autoimmune hepatitis, celiac disease and type 1 diabetes mellitus) in cSLE. To assess outcomes, we studied measures of lupus disease activity, complications, and patient’s quality of life (QoL). Comparisons by PA status were made using chi-square, Fisher’s exact test, two-sample t-tests, Wilcoxon rank sum tests, and mixed effects models as appropriate. Results 1285 patients met the American College of Rheumatology criteria for SLE. Of those, 388 (30%) had data on comorbidity. The prevalence of PA was 8.8%. Patients with PA reported more hospitalizations and aggressive immunotherapy use. SLEDAI and PGA scores improved over time, but did not differ by PA status. No significant differences were found in QoL measures or their trajectory over time by PA status. Conclusion In cSLE, PA is associated with more hospitalizations and aggressive immunotherapy use. Although lupus disease activity improved over time, patients' QoL neither improved over time nor differed by having other autoimmune disease. Prospective, case-control, long-term follow-up studies on cSLE are needed to validate our results. MeSH Key Indexing Terms Pediatric systemic lupus erythematosus; Autoimmune diseases; Outcome assessment
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Ferradás, Rubén R., Santiago Marqués-González, Henrry M. Osorio, et al. "Low variability of single-molecule conductance assisted by bulky metal–molecule contacts." RSC Advances 6, no. 79 (2016): 75111–21. http://dx.doi.org/10.1039/c6ra15477h.
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Cantelar Rodríguez, Francisco. "Directrices para Ia redacción del Catálogo de Ia CSLE." Revista Española de Derecho Canónico 55, no. 145 (1998): 755–59. http://dx.doi.org/10.36576/summa.5994.
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Wang, Youwu, Jingchuan Zhang, Guangchao Kong, and Hai Lin. "Fine mapping the QTLqFS-chr.23, using a CSIL." Plant Breeding 135, no. 4 (2016): 492–98. http://dx.doi.org/10.1111/pbr.12381.
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Silva, Marco F., Mariana P. Ferriani, Maria T. Terreri, et al. "A Multicenter Study of Invasive Fungal Infections in Patients with Childhood-onset Systemic Lupus Erythematosus." Journal of Rheumatology 42, no. 12 (2015): 2296–303. http://dx.doi.org/10.3899/jrheum.150142.
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Objective.To study the prevalence, risk factors, and mortality of invasive fungal infections (IFI) in patients with childhood-onset systemic lupus erythematosus (cSLE).Methods.A retrospective multicenter cohort study was performed in 852 patients with cSLE from 10 pediatric rheumatology services. An investigator meeting was held and all participants received database training. IFI were diagnosed according to the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group Consensus Group criteria (proven, probable, and possible). Also evaluated were demographic, clinical, and laboratory data, and disease activity [SLE Disease Activity Index 2000 (SLEDAI-2K)], cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), treatment, and outcomes.Results.IFI were observed in 33/852 patients (3.9%) with cSLE. Proven IFI was diagnosed in 22 patients with cSLE, probable IFI in 5, and possible IFI in 6. Types of IFI were candidiasis (20), aspergillosis (9), cryptococcosis (2), and 1 each disseminated histoplasmosis and paracoccidioidomycosis. The median of disease duration was lower (1.0 vs 4.7 yrs, p < 0.0001) with a higher current SLEDAI-2K [19.5 (0–44) vs 2 (0–45), p < 0.0001] and current prednisone (PRED) dose [50 (10–60) vs 10 (2–90) mg/day, p < 0.0001] in patients with IFI compared with those without IFI. The frequency of death was higher in the former group (51% vs 6%, p < 0.0001). Logistic regression analysis revealed that SLEDAI-2K (OR 1.108, 95% CI 1.057–1.163, p < 0.0001), current PRED dose (OR 1.046, 95% CI 1.021–1.071, p < 0.0001), and disease duration (OR 0.984, 95% CI 0.969–0.998, p = 0.030) were independent risk factors for IFI (R2 Nagelkerke 0.425).Conclusion.To our knowledge, this is the first study to characterize IFI in patients with cSLE. We identified that disease activity and current glucocorticoid use were the main risk factors for these life-threatening infections, mainly in the first years of disease course, with a high rate of fatal outcome.
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Yang, Yelin, Sathish Kumar, Lily Siok Hoon Lim, Earl D. Silverman, and Deborah M. Levy. "Risk Factors for Symptomatic Avascular Necrosis in Childhood-onset Systemic Lupus Erythematosus." Journal of Rheumatology 42, no. 12 (2015): 2304–9. http://dx.doi.org/10.3899/jrheum.150464.
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Objective.To examine the frequency and risk factors for symptomatic avascular necrosis (AVN) in childhood-onset systemic lupus erythematosus (cSLE).Methods.A single-center, nested, matched, case-control design was used. There were 617 patients with cSLE followed at the Hospital for Sick Children (SickKids) Lupus Clinic between July 1982 and June 2013 included in the study. The AVN cohort consisted of 37 patients identified with clinical findings of symptomatic AVN and diagnosis was confirmed by 1 or more imaging modalities. Three controls were matched to each patient with AVN by date and age at diagnosis. Baseline clinical, laboratory, and treatment characteristics were compared between patients with AVN and controls by univariable analyses and if statistically significant, were included in a multivariable logistic regression model.Results.A total of 37/617 patients (6%) developed symptomatic AVN in 91 joints during followup at SickKids. The mean duration to disease was 2.3 years. The hip was the most commonly involved joint (26/37, 70%). Compared with the matched non-AVN cohort, patients with AVN had a higher incidence of central nervous system (CNS) involvement and nephritis, required greater cumulative prednisone (PRED) from cSLE diagnosis to AVN, received a greater maximal daily PRED dose, and had more frequent use of pulse methylprednisolone therapy. Multivariable regression analysis confirmed major organ involvement (CNS disease and/or nephritis) and maximal daily PRED dose as significant predictors of symptomatic AVN development.Conclusion.Patients with cSLE with severe organ involvement including nephritis and CNS disease and higher maximal daily dose of PRED are more likely to develop symptomatic AVN.
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Correll, Colleen K., Mitali Dave, Anne F. Paul, et al. "Identifying Research Priorities among Patients and Families of Children with Rheumatic Diseases Living in the United States." Journal of Rheumatology 47, no. 12 (2020): 1800–1806. http://dx.doi.org/10.3899/jrheum.190934.
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ObjectiveTo improve the quality and participation in pediatric rheumatology research, patient-prioritized studies should be emphasized. We collaborated with United States–based pediatric rheumatology advocacy organizations to survey patients and caregivers of children with rheumatic diseases to identify what research topics were most important to them.MethodsWe conducted Web-based surveys and focus groups (FG) of patients and caregivers of children with juvenile myositis (JM), juvenile arthritis (JA), and childhood-onset systemic lupus erythematosus (cSLE). Surveys were emailed to listservs and posted to social media sites of JM, JA, and cSLE patient advocacy organizations. An initial survey asked open-ended questions about patient/caregiver research preferences. Responses were further characterized through FG. A final ranking survey asked respondents to rank from a list of research themes the 7 most important to them.ResultsThere were 365 JM respondents, 44 JA respondents, and 32 cSLE respondents to the final ranking survey. The top research priority for JM was finding new treatments, and for JA and cSLE, the priority was understanding genetic/environmental etiology. The 3 prioritized research themes common across all disease groups were medication side effects, disease flare, and disease etiology.ConclusionPatient-centered research prioritization is recognized as valuable in conducting high-quality research, yet there is a paucity of data describing patient/family preferences, especially in pediatrics. We used multimodal methodologies to assess current patient/caregiver research priorities to help frame the agenda for the pediatric rheumatology research community. Patients and caregivers from all surveyed disease groups prioritized the study of medication side effects, disease flares, and disease etiology.
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De Bonis, Maria Carmela. "Remarks on two integral operators and numerical methods for CSIE." Journal of Computational and Applied Mathematics 260 (April 2014): 117–34. http://dx.doi.org/10.1016/j.cam.2013.09.063.
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Bortolini, Maria Fernanda Ferraz, Vitória Peres Pereira, Thiago AF Gomes Dos Santos, Renato Nisihara, and Thelma L. Skare. "Systemic lupus erythematosus in children and adults: A retrospective study in Brazilian patients." Lupus 30, no. 7 (2021): 1197–202. http://dx.doi.org/10.1177/09612033211010330.
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Background Systemic lupus erythematosus (SLE) may have a different serological and clinical profile according to age of disease onset. Aim To compare clinical presentation and serological data from patients with SLE onset in childhood (cSLE) with disease onset in adulthood (aSLE) in a sample of Brazilian patients. Methods Retrospective study of 614 SLE patients from a single Rheumatology Unit from Brazil: 77 (12.5%) cSLE and 537 (87.4%) aSLE. Clinical and serological data were obtained from the charts. Comparisons of cSLE with aSLE in general and according to patient’s gender were made. Results The comparison of whole sample showed that children had more malar rash (p = 0.04), seizures (p < 0.0001), psychosis (p = 0.02), glomerulonephritis (p = 0.001), anti-dsDNA (p = 0.008), anticardiolipin IgM (p = 0.04) but less discoid lesions (p = 0.01), anti-Ro (p < 0.0001) and anti-La antibodies (p = 0.007). When only the male sample was compared, no differences in glomerulonephritis and anti-dsDNA frequencies were found. Conclusion Children had a higher frequency of severe manifestations (glomerulonephritis and central nervous system) than adults. The difference in glomerulonephritis occurrence disappeared when only males were compared.
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Eller, Christina, Gerald Kehr, Constantin G. Daniliuc, and Gerhard Erker. "Unusual product formation in a 1,1-carboboration reaction." Chemical Communications 51, no. 40 (2015): 8436–38. http://dx.doi.org/10.1039/c5cc00734h.
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Nguyen, P., S. Barraud, M. Casse, et al. "-Gate Nanowire P-FET with cSiGe Channel Epitaxied on Strained-SOI Substrates." ECS Transactions 75, no. 8 (2016): 59–65. http://dx.doi.org/10.1149/07508.0059ecst.
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Podolean, Iunia, Christopher Hardacre, Peter Goodrich, et al. "Chiral supported ionic liquid phase (CSILP) catalysts for greener asymmetric hydrogenation processes." Catalysis Today 200 (February 2013): 63–73. http://dx.doi.org/10.1016/j.cattod.2012.06.020.
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Wahezi, Dawn M. "Proliferative nephritis in childhood-onset systemic lupus erythematosus: current therapeutic approaches and future perspectives." F1000Research 4 (May 27, 2015): 128. http://dx.doi.org/10.12688/f1000research.6533.1.
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Renal involvement occurs in 50-75% of children with childhood-onset systemic lupus erythematosus (cSLE). Proliferative lupus nephritis (LN) represents the most common pattern of renal involvement in cSLE. Despite aggressive treatment, progression to end stage renal disease can occur in up to 5-10% of children. Over the last 2 decades, tremendous advancements have been made in the treatment of pediatric LN. Special considerations in children need to address the impact of disease and therapy on both physical and psychological growth and development. This review will focus on pivotal clinical trials in the treatment of proliferative LN, with a focus on pediatric data when available.
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Chighizola, C., I. Pontikaki, S. Costi, et al. "POS1308 PREDICTORS OF DISEASE PROGRESSION IN A MONOCENTRIC COHORT OF 100 PATIENTS WITH CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS." Annals of the Rheumatic Diseases 80, Suppl 1 (2021): 936.1–936. http://dx.doi.org/10.1136/annrheumdis-2021-eular.2051.
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Background:Childhood-onset systemic lupus erythematosus (cSLE) accounts for 10-20% of overall cases, usually presenting in early adolescence. SLE in children is characterized by a severe clinical course.Objectives:This study describes the evolution of clinical manifestations of cSLE, with the aims of i) identifying biomarkers predictive of disease progression and ii) assessing the effects of treatment on disease course.Methods:Laboratory and clinical data of cSLE patients followed in our Paediatric Rheumatology Unit were retrospectively collected at diagnosis and during follow-up. Continuous data were expressed as median (interquartile range [IQR]) and categorical data as percentages. The association between categorical variables was assessed by chi-squared test, the correlation between variables was tested by Pearson’s test. Univariate linear regression analyses were performed to investigate the relationship between the rate of new disease manifestations at follow-up and candidate predictors. Statistical analysis was performed using GraphPadPrism v6. P values <0.05 were regarded statistically significant.Results:One-hundred patients (89% of female gender) with cSLE were included in this study, with a median age at disease onset of 13 years (10.5-15). Clinical details are presented in Table 1. Complement levels were reduced in 71 patients (C3 in 62 and C4 in 65). At a median follow-up of 118 months (57-239), the disease progressed in 93 patients, with a median of two new manifestations per patient (1-3). No correlation emerged between the number of new disease manifestations and both age at diagnosis and disease duration. Among serological biomarkers, a reduction in complement fractions at diagnosis emerged as the only predictor of new clinical manifestations due to cSLE (p=0.013 for low C3 and p=0.0004 for low C4). Among the several different pharmacological tools, hydroxychloroquine (HCQ, p=0.021, 95% confidence interval (CI) 0.0084-0.1007), azathioprine (AZA, p=0.0217, 95%CI 0.013-0.1703) and cyclophosphamide (CTX, p=0.0305, 95%CI 0.013-0.170) were identified as protective.At follow-up, patients most commonly developed new haematological and cutaneous involvements, which were diagnosed in 43 and 11 patients, respectively. A trend towards statistical significance emerged for low C4 levels to predict new haematological involvement at follow-up (p=0.064, chi-squared: 3.42). Differently, positivity for antibodies against dsDNA emerged as the only predictor of the onset of cutaneous manifestations during follow-up (p=0.022, chi-squared: 7.62). Low C3 levels approached statistical significance in the prediction of skin involvement (p=0.058, chi-squared 5.68).Conclusion:According to the data from our monocentric cohort of 100 patients, complement and anti-dsDNA antibodies are the most accurate tools to predict disease progression in cSLE. HCQ, AZA and CTX reduce the rate of disease progression.Table 1.Clinical manifestations, laboratory features and treatment details of recruited patients at diagnosis.Number of patientsSkin manifestations44Haematological involvement51Lupus nephritis16Musculoskeletal involvement72Serositis14Neuropsychiatric involvement6Anti-nuclear antibodies90Anti-Ro antibodies11Anti-Sm antibodies13Anti-dsDNA antibodies67Anti-phospholipid antibodies29Hydroxychloroquine89Azathioprine40Cyclophosphosphamide29Micophenolate mofetil13Cyclosporine15Rituximab1Belimumab2Steroids91Disclosure of Interests:None declared
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Haro, Sara L., Erica F. Lawson, and Aimee O. Hersh. "Disease activity and health-care utilization among young adults with childhood-onset lupus transitioning to adult care: data from the Pediatric Lupus Outcomes Study." Lupus 29, no. 10 (2020): 1206–15. http://dx.doi.org/10.1177/0961203320938868.
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Background Individuals with childhood-onset systemic lupus erythematosus (cSLE) must transfer from pediatric to adult care. The goal of this study was to examine disease activity and health-care utilization among young adults with cSLE who are undergoing or have recently completed the transfer to adult care. Methods The Pediatric Lupus Outcomes Study (PLOS) is a prospective longitudinal cohort study of young adults aged 18–30 diagnosed with cSLE. We conducted a cross-sectional analysis comparing 47 participants under the care of pediatric rheumatologists to 38 who had completed transfer to adult care. Demographics, disease manifestations, health- care utilization and transition readiness were compared between groups. Results Those in the post-transfer group had significantly lower medication usage and were less likely to have seen a rheumatologist in the past year. Disease manifestations, flare rates, and hospitalizations were similar between groups. Nearly a quarter of patients who had transferred to adult care reported difficulties with the process. Conclusion Post-transfer patients had lower health-care utilization as evidenced by less medication usage and lack of rheumatology follow-up, in spite of the fact that disease activity was similar in both groups. Future studies will assess longitudinal changes in disease activity and damage in this population.
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Moorthy, L. N., M. E. Baldino, V. Kurra, et al. "Relationship between health-related quality of life, disease activity and disease damage in a prospective international multicenter cohort of childhood onset systemic lupus erythematosus patients." Lupus 26, no. 3 (2016): 255–65. http://dx.doi.org/10.1177/0961203316659546.
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Previously, we described associations between health-related quality of life (HRQOL) and disease-related factors among childhood onset systemic lupus erythematosus (cSLE) patients. Here we determined the relationship between HRQOL, disease activity and damage in a large prospective international cohort of cSLE. We compared HRQOL, disease activity and disease damage across different continents and examined the relationship between children's and parents' assessments of HRQOL. Patients with cSLE and their parents completed HRQOL measures at enrollment and ≥4 follow-up visits. Physicians assessed disease activity and damage. The multinational cohort ( n = 467) had relatively low disease activity and damage. Patient and parent HRQOL scores were significantly correlated. Asian and European patients had the highest HRQOL, while South and North American patients had lower HRQOL scores. Renal, CNS, skin and musculoskeletal systems exhibited the highest levels of damage. North and South American and Asian patients were more likely to have disease damage and activity scores above median values, compared with Europeans. Asians were more likely to use cyclophosphamide/rituximab. Female gender, high disease activity and damage, non-White ethnicity, and use of cyclophosphamide and/rituximab were related to lower HRQOL. HRQOL domain scores continue to emphasize that SLE has widespread impact on all aspects of children's and parents' lives.
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Hill, Anthony F., Richard A. Manzano, and Jas S. Ward. "Synthesis and reactivity of selenium functionalised allylidynes and propargylidynes." Dalton Transactions 47, no. 41 (2018): 14621–29. http://dx.doi.org/10.1039/c8dt02370k.
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The reactions of the trimethylsilylpropargylidyne [W(CCCSiMe<sub>3</sub>)(CO)<sub>2</sub>(Tp*)] (1: Tp* = hydrotris(dimethylpyrazolyl)borate) towards selenium centred reagents when treated with tetrabutylammonium fluoride (TBAF) were explored in order to prepare alkynylselenolato propargylidynes, e.g., [W(CCCSePh)(CO)<sub>2</sub>(Tp*)].
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Kohut, S. Ahola, TS Williams, J. Jayanthikumar, et al. "Depressive symptoms are prevalent in childhood-onset systemic lupus erythematosus (cSLE)." Lupus 22, no. 7 (2013): 712–20. http://dx.doi.org/10.1177/0961203313488840.
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