Demographic, clinical, and treatment characteristics of the juvenile primary fibromyalgia syndrome cohort enrolled in the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry.

BackgroundTo describe the demographic, clinical, and treatment characteristics of youth diagnosed with juvenile primary fibromyalgia syndrome (JPFS) who are seen in pediatric rheumatology clinics.MethodsInformation on demographics, symptoms, functioning, and treatments recommended and tried were obtained on patients with JPFS as part of a multi-site patient registry (the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry). Data were summarized using descriptive statistics. In a subset of patients completing registry follow-up visits, changes in symptoms, pain, and functioning were evaluated using growth modeling.ResultsOf the 201 patients with JPFS enrolled in the registry, most were Caucasian/White (85%), non-Hispanic (83%), and female (84%). Ages ranged from 9 to 20 years (M = 15.4 + 2.2). The most common symptoms reported were widespread musculoskeletal pain (91%), fatigue (84%), disordered sleep (82%), and headaches (68%). Pain intensity was rated as moderate to severe (M = 6.3 + 2.4/10). Scores on measures of functioning indicated mild to moderate impairment, with males observed to report significantly greater impairments. For the 37% of the initial cohort having follow-up data available, indicators of function and well-being were found to either worsen over time or remain relatively unchanged.ConclusionsThe symptoms of JPFS remained persistent and disabling for many patients treated by pediatric rheumatologists. Further study appears warranted to elucidate gender differences in the impact of JPFS symptoms. Work also is needed to identify accessible and effective outpatient treatment options for JPFS that can be routinely recommended or implemented by pediatric rheumatology providers

IVOA Recommendation: Resource Metadata for the Virtual Observatory Version 1.12

An essential capability of the Virtual Observatory is a means for describing what data and computational facilities are available where, and once identified, how to use them. The data themselves have associated metadata (e.g., FITS keywords), and similarly we require metadata about data collections and data services so that VO users can easily find information of interest. Furthermore, such metadata are needed in order to manage distributed queries efficiently; if a user is interested in finding x-ray images there is no point in querying the HST archive, for example. In this document we suggest an architecture for resource and service metadata and describe the relationship of this architecture to emerging Web Services standards. We also define an initial set of metadata concepts

Assessment of chronic postsurgical pain after knee replacement : a systematic review

Objective: Approximately 20% of patients experience chronic pain after total knee replacement (TKR), yet there is no consensus about how best to assess such pain. This systematic review aimed to identify measures used to characterise chronic pain after TKR. Methods: MEDLINE, Embase, PsycINFO, Cochrane Library and CINAHL databases were searched for research articles published in all languages from January 2002- November 2011. Articles were eligible for inclusion if they assessed knee pain at a minimum of 3-months after TKR, yielding a total of 1,164 articles. Data extracted included study design, country, timings of assessments, and outcome measures containing pain items. Outcome measures were compared with domains recommended by IMMPACT for inclusion in assessment of chronic pain related outcomes within clinical trials. Temporal trends were also explored. Results: The review found use of a wide variety of composite and single-item measures, with the American Knee Society Score most common. Many measures used in published studies do not capture the multi-dimensional nature of pain recommended by IMMPACT; of those commonly used, the WOMAC and Oxford Knee Score are most comprehensive. Geographical trends were evident, with nation-specific preferences for particular measures. A recent reduction in use of some clinically-administered tools was accompanied by increased use of patient-reported outcome measures. Conclusion: There was wide variation in methods of pain assessment alongside nation-specific preferences and changing temporal trends in pain assessment after TKR. Standardisation and improvements in assessment is needed to enhance the quality of research and facilitate the establishment of a core outcome set

Impact of early coronary artery bypass graft in an unselected acute coronary syndrome patient population

BACKGROUND: Performance of coronary artery bypass graft (CABG) during an acute coronary syndrome (ACS) is mainly used in high-risk patients. Although potentially life-saving, patients undergoing early CABG are traditionally associated with a worse outcome than those not requiring CABG. Is this really true in an unselected ACS population? The aim of this study was to evaluate, in an ACS population, if the performance of CABG during the index hospitalization influences in-hospital outcome. METHODS AND RESULTS: Retrospective analysis of a nationwide database of 12,988 ACS patients admitted since 2002. Of those, 267 patients underwent CABG during the index hospitalization (group A) and 12,721 did not (group B). Group B patients were further divided in 2 subgroups: those submitted to percutaneous coronary interventions (PCI) (group B1; n=3948) during the index hospitalization and those not submitted to mechanical revascularization (group B2; n =8773). Patients from group A more frequently had diabetes, hypercholesterolemia, hypertension, and previous angina; they were also more often on cardiovascular medication before admission. Patients that underwent CABG were more often in Killip class IV at admission (4.8% versus 1.4% versus 2.0%); they also received more nitrates and catecholamines. Left ventricular function was better in group B1. Group A patients were more often on mechanical ventilation and intra-aortic pump and they had more in-hospital complications (31.1% versus 18.7% versus 17.3%), namely recurrent angina, re-infarction, and mechanical complications. They had a more severe coronary anatomy and the culprit lesion was more frequently on the left main (7.7% versus 0.5% versus 2.2%). However, their in-hospital mortality was significantly lower (1.1% versus 2.2% versus 6.8%; P<0.001). Multivariate analysis showed that performance of early CABG was an independent predictor of lower mortality (odds ratio of 0.12), as were the use of low-molecular-weight heparins, beta-blockers, and angiotensin-converting enzyme inhibitors. CONCLUSIONS: In unselected patients admitted for ACS, performance of early CABG, despite being performed in higher-risk patients, is associated with very low in-hospital mortality, even when compared with the mortality of lower-risk population not submitted to early CABG. Therefore, early performance of this procedure should be considered more often in eligible patients

IVOA Recommendation: VOResource: an XML Encoding Schema for Resource Metadata Version 1.03

This document describes an XML encoding standard for IVOA Resource Metadata, referred to as VOResource. This schema is primarily intended to support interoperable registries used for discovering resources; however, any application that needs to describe resources may use this schema. In this document, we define the types and elements that make up the schema as representations of metadata terms defined in the IVOA standard, Resource Metadata for the Virtual Observatory [Hanicsh et al. 2004]. We also describe the general model for the schema and explain how it may be extended to add new metadata terms and describe more specific types of resources

Perinatal insults and neurodevelopmental disorders may impact Huntington's disease age of diagnosis

Introduction: The age of diagnosis of Huntington's disease (HD) varies among individuals with the same HTT CAG-repeat expansion size. We investigated whether early-life events, like perinatal insults or neurodevelopmental disorders, influence the diagnosis age. Methods: We used data from 13,856 participants from REGISTRY and Enroll-HD, two large international multicenter observational studies. Disease-free survival analyses of mutation carriers with an HTT CAG repeat expansion size above and including 36 were computed through Kaplan-Meier estimates of median time until an HD diagnosis. Comparisons between groups were computed using a Cox proportional hazard survival model adjusted for CAG-repeat expansion length. We also assessed whether the group effect depended on gender and the affected parent. Results: Insults in the perinatal period were associated with an earlier median age of diagnosis of 45.00 years (95%CI: 42.07–47.92) compared to 51.00 years (95%CI: 50.68–51.31) in the reference group, with a CAG-adjusted hazard ratio of 1.61 (95%CI: 1.26–2.06). Neurodevelopmental disorders were also associated with an earlier median age of diagnosis than the reference group of 47.00 years (95% CI: 43.38–50.62) with a CAG-adjusted hazard ratio of 1.42 (95%CI: 1.16–1.75). These associations did not change significantly with gender or affected parent. Conclusions: These results, derived from large observational datasets, show that perinatal insults and neurodevelopmental disorders are associated with earlier ages of diagnosis of magnitudes similar to the effects of known genetic modifiers of HD. Given their clear temporal separation, these early events may be causative of earlier HD onset, but further research is needed to prove causation

Clinical phenotypes and biologic treatment use in juvenile dermatomyositis-associated calcinosis

Abstract Background Few risk factors have been identified for the development of calcinosis among patients with Juvenile Dermatomyositis, and currently no clinical phenotype has been associated with its development. We analyzed a large database of patients to further elucidate any relationships among patients with and without calcinosis. Method The CARRA legacy registry recruited pediatric rheumatology patients from 55 centers across North America from 2010 through 2014, including over 650 subjects with Juvenile Dermatomyositis. We compared the demographic characteristics, clinical disease features and treatment histories of those with and without calcinosis using univariate and multivariate logistic regression. Results Of the 631 patients included in the analysis, 84 (13%) had a current or prior history of calcinosis. These patients were statistically more likely to have longer durations of disease prior to diagnosis and treatment, have lipodystrophy and joint contractures, and to have received intravenous immune globulin or rituximab as treatments. Conclusions Calcinosis is found more often in patients with prolonged active disease, severe disease, and certain clinical features such as lipodystrophy and joint contractures. When these factors are combined with other known associations and predictors, groups of at-risk patients can be more effectively identified, treated and studied to improve overall outcomes