60 research outputs found
Student Project: Replication of Author, Author, & Author (201X, JDM, Study X) - Template
No full textThis project is an independent replication of Author, Author, & Author (201X, study X) conducted as part of the Hagen Cumulative Science Project
HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis-scleroderma overlap
Get PDFObjectives. To investigate a large cohort of children with juvenile dermatomyositis (JDM), and those with JDM-scleroderma (JDM-SSc) overlap, using detailed serological analysis, HLA class II genotyping and clinical characterization. Methods. Children (114) with JDM were recruited, and clinical data collected, through the JDM National Registry and Repository (UK and Ireland). Sera were assayed for ANA using standard immunofluorescence techniques and specific antibodies characterized using ELISA, immunodiffusion and radioimmunoprecipitation. Patients and controls (n = 537) were genotyped at the HLA-DRB1 and DQB1 loci, and then the DQA1 locus data was derived. Results. Over 70% of the patients were ANA-positive. Clear differences in serological and genetic data were demonstrated between JDM and JDM-SSc overlap groups. Strong associations were seen for HLA-DRB1-03 (all cases vs controls, Pcorr = 0.02; JDM-SSc vs controls, Pcorr = 0.001) and HLA-DQA1*05 (all cases vs controls, Pcorr = 0.01; JDM-SSc vs controls, Pcorr = 0.005). The frequency of the HLA-DRB1*03-DQA1*05-DQB1*02 haplotype was significantly increased in the JDM-SSc (P = 0.003) and anti-PM-Scl antibody (P = 0.002) positive groups. All anti-U1-RNP antibody-positive patients had at least one copy of HLA-DRB1*04-DQA1*03-DQB1*03 haplotype. Associations were observed between serology and specific clinical features. Conclusions. We present clinical data, HLA genotyping and serological profiling on a large cohort of JDM patients and a carefully characterized subset of patients with JDM-SSc overlap. The results confirm known HLA associations and extend the knowledge by stratification of data in serological and clinical subgroups. In the future, a combination of serological and genetic typing may allow for better prediction of clinical course and disease subtype in JDM. © The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved
Standardized nailfold capillaroscopy in children with rheumatic diseases:a worldwide study
No full textObjectives: To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods: In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results: A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. Conclusions: This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD. VC The Author(s) 2022.</p
Standardized nailfold capillaroscopy in children with rheumatic diseases:a worldwide study
No full textObjectives: To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods: In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results: A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. Conclusions: This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD. VC The Author(s) 2022.</p
Standardized nailfold capillaroscopy in children with rheumatic diseases:a worldwide study
No full textObjectives: To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods: In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results: A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. Conclusions: This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD. VC The Author(s) 2022.</p
Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis
No full textObjectives. Myositis-specific autoantibodies (MSAs) may define homogeneous clinical subsets of adult patients with dermatomyositis (DM). Recently, there have been descriptions of novel autoantibodies in DM. This study was conducted to establish the clinical significance of anti-p155/140 autoantibodies in juvenile DM (JDM). Methods. The first 116 children recruited to the JDM National Registry and Repository (UK and Ireland) were studied. Comprehensive clinical features were recorded and sera screened for anti-p155/140 autoantibodies using radio-immunoprecipitation. Sera from adults with DM (n=20), PM (n=25), SSc (n=150), SLE (n=40) and healthy subjects (n=50) were used for comparison. Immunodepletion experiments were used to establish whether the p155/140 kDa targets recognized by JDM sera were the same as adult DM sera. Results. Twenty-seven out of 116 (23%) JDM cases were positive for anti-p155/140 in comparison with 6/20 (30%) adults with DM. Immunodepletion confirmed that the 155/140 kDa proteins recognized by JDM and adult DM sera were the same targets. All other adult control sera were negative for anti-p155/140 autoantibodies. There was a higher frequency of males in the anti-p155/140-positive JDM group (P=0.02). JDM patients with anti-p155/140 autoantibodies had significantly more cutaneous involvement including Gottron's papules (P=0.003), ulceration (P=0.005) and oedema (P=0.013). The distribution of skin lesions was more extensive particularly periorbitally (P=0.014) and over the small (P≥0.001) and large joints (P=0.003). Conclusions. Anti-p155/140 autoantibodies are clinically significant in JDM and may define a clinical subset in terms of disease severity and outcome. The same autoantigen target is detected in adult DM patients. © The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved
Graph realizations constrained by skeleton graphs
No full textIn 2008 Amanatidis, Green and Mihail introduced the Joint Degree Matrix (JDM) model to capture the fundamental difference in assortativity of networks in nature studied by the physical and life sciences and social networks studied in the social sciences. In 2014 Czabarka proposed a direct generalization of the JDM model, the Partition Adjacency Matrix (PAM) model. In the PAM model the vertices have specified degrees, and the vertex set itself is partitioned into classes. For each pair of vertex classes the number of edges between the classes in a graph realization is prescribed. In this paper we apply the new skeleton graph model to describe the same information as the PAM model. Our model is more convenient for handling problems with low number of partition classes or with special topological restrictions among the classes. We investigate two particular cases in detail: (i) when there are only two vertex classes and (ii) when the skeleton graph contains at most one cycle.Discrete Mathematics and Optimizatio
Adaptive information search and judgment strategies in solitary and competitive tasks
Get PDFA substantial body of judgment and decision-making research focuses on decisions made under risk, where all relevant option outcome and probability information is known a priori. However, most real-world decision tasks are made under uncertainty, where such population- level information is unknown. Against this background, how can, do, and should organisms obtain and use information in order to improve their judgments and decisions under uncertainty? This dissertation addresses these questions in two distinct domains: external information search in competitive tasks (papers 1 and 2) and internal search in the context of the inner-crowd (papers 3 and 4). In paper 1, we develop a new paradigm called the Competitive Sampling Game (CSG) to study how organisms adjust search in the presence of both natural uncertainty (i.e., gamble parameters) and social uncertainty (i.e., behavior of others). The paradigm produces simulation and empirical results showing that organisms should and do dramatically reduce search in the presence of competition to almost minimal levels. In paper 2, we expand on the initial results of the CSG to show how different levels of competition drive the evolution of decision strategies. In a second domain, we address how people can improve their judgments by harnessing a diverse inner-crowd using dialectical bootstrapping. In paper 3, we apply dialectical bootstrapping to a Bayesian reasoning paradigm to show how dialectical instructions induce strategy change and how people can become more Bayesian by averaging biased non-Bayesian judgments in their inner-crowd. In paper 4, we apply the inner-crowd to a cue-based estimation task and model the effects of different estimation strategies on final estimates and confidence. Our results suggest that people can use their confidence judgments to outperform the simple average of their inner-crowd. Moreover, dialectical bootstrapping increases these effects
Cerebral Vasculities Associated with Shingles
Get PDFShingles is a common manifestation of infection with herpes zoster virus (more correctly varicella-zoster virus) in middle-aged or elderly people. We describe three patients who developed brain stem encephalitis and cerebral vasculitis due to infection with this agent during a 12-month period
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