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The autoimmune disease lupus erythematosus (lupus) is characterized by photosensitivity, where even ambient ultraviolet radiation (UVR) exposure can lead to development of inflammatory skin lesions. We have previously shown that Langerhans cells (LCs) limit keratinocyte apoptosis and photosensitivity via a disintegrin and metalloprotease 17 (ADAM17)-mediated release of epidermal growth factor receptor (EGFR) ligands and that LC ADAM17 sheddase activity is reduced in lupus. Here, we sought to understand how the lupus skin environment contributes to LC ADAM17 dysfunction and, in the process, differentiate between effects on LC ADAM17 sheddase function, LC ADAM17 expression, and LC numbers. We show through transcriptomic analysis a shared IFN-rich environment in non-lesional skin across human lupus and three murine models: MRL/lpr, B6.Sle1yaa, and imiquimod (IMQ) mice. IFN-I inhibits LC ADAM17 sheddase activity in murine and human LCs, and IFNAR blockade in lupus model mice restores LC ADAM17 sheddase activity, all without consistent effects on LC ADAM17 protein expression or LC numbers. Anti-IFNAR-mediated LC ADAM17 sheddase function restoration is associated with reduced photosensitive responses that are dependent on EGFR signaling and LC ADAM17. Reactive oxygen species (ROS) is a known mediator of ADAM17 activity; we show that UVR-induced LC ROS production is reduced in lupus model mice, restored by anti-IFNAR, and is cytoplasmic in origin. Our findings suggest that IFN-I promotes photosensitivity at least in part by inhibiting UVR-induced LC ADAM17 sheddase function and raise the possibility that anifrolumab ameliorates lupus skin disease in part by restoring this function. This work provides insight into IFN-I-mediated disease mechanisms, LC regulation, and a potential mechanism of action for anifrolumab in lupus.

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Postgraduate medical education is an essential societal enterprise that prepares highly skilled physicians for the health workforce. In recent years, PGME systems have been criticized worldwide for problems with variable graduate abilities, concerns about patient safety, and issues with teaching and assessment methods. In response, competency based medical education approaches, with an emphasis on graduate outcomes, have been proposed as the direction for 21st century health profession education. However, there are few published models of large-scale implementation of these approaches. We describe the rationale and design for a national, time-variable competency-based multi-specialty system for postgraduate medical education called Competence by Design. Fourteen innovations were bundled to create this new system, using the Van Melle Core Components of competency based medical education as the basis for the transformation. The successful execution of this transformational training system shows competency based medical education can be implemented at scale. The lessons learned in the early implementation of Competence by Design can inform competency based medical education innovation efforts across professions worldwide.

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Competency based medical education is being adopted around the world. Accreditation plays a vital role as an enabler in the adoption and implementation of competency based medical education, but little has been published about how the design of an accreditation system facilitates this transformation. The Canadian postgraduate medical education environment has recently transitioned to an outcomes-based accreditation system in parallel with the adoption of competency based medical education. Using the Canadian example, we characterize four features of an accreditation system that can facilitate the implementation of competency based medical education: theoretical underpinning, quality focus, accreditation standards, and accreditation processes. Alignment of the underlying educational theories within the accreditation system and educational paradigm drives change in a consistent and desired direction. An accreditation system that prioritizes quality improvement over quality assurance promotes educational system development and progressive change. Accreditation standards that achieve the difficult balance of being sufficiently detailed yet flexible foster a high fidelity of implementation without stifling innovation. Finally, accreditation processes that recognize the change process, encourage program development, and are not overly punitive all enable the implementation of competency based medical education. We also discuss the ways in which accreditation can simultaneously hinder the implementation of this approach. As education bodies adopt competency based medical education, particular attention should be paid to the role that accreditation plays in successful implementation.

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BACKGROUND/OBJECTIVE: The importance of patient-reported outcomes, like the Patient-Reported Outcomes Measurement Information System (PROMIS) measures, is increasingly recognized both in clinical care and in research. While "short forms" have been studied in juvenile idiopathic arthritis (JIA), study of PROMIS computer adaptive tests (CATs) in JIA is limited. This cross-sectional study evaluates whether PROMIS CATs correlate with disease activity in patients with JIA. METHODS: A convenience sample of patients with JIA (N = 44) was recruited from a single center. Patients and parents completed pediatric and parent proxy PROMIS CATs. Disease activity was evaluated using the Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71) and the Childhood Health Assessment Questionnaire (CHAQ). Correlation of the CAT T scores with disease activity was assessed using Spearman correlation coefficients. RESULTS: Forty-four of 80 eligible subjects (29 patients and 15 parents) completed all or some PROMIS CATs. Pain interference and mobility CATs correlated moderately with JADAS-71. Nearly all correlations with the JADAS-71 were weakened when the patient global was removed. Pain interference, mobility, and fatigue were strongly correlated with the CHAQ. Among parent proxy CATs, only mobility and depressive symptoms correlated strongly with the CHAQ. CONCLUSIONS: Only pain interference and mobility PROMIS CATs showed strong correlation with standard disease activity measures in JIA, and nearly all correlations were weakened when the patient global was removed. Correlations of the CATs with the CHAQ were stronger than correlations with the JADAS-71, indicating that although the CHAQ is no longer routinely used it may be a better measure of health-related quality of life in routine clinical care.

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BACKGROUND: Accreditation is a key feature of many medical education systems, helping to ensure that programs teach and assess learners according to applicable standards, provide optimal learning environments, and produce professionals who are competent to practise in challenging and evolving health care systems. Although most medical education accreditation systems apply similar standards domains and process elements, there can be substantial variation among accreditation systems at the level of design and implementation. A discussion group at the 2013 World Summit on Outcomes-Based Accreditation examined best practices in health professional education accreditation systems and identified that the literature examining the effectiveness of different approaches to accreditation is scant. Although some frameworks for accreditation design do exist, they are often specific to one phase of the medical education continuum. MAIN TEXT: This paper attempts to define a framework for the operational design of medical education accreditation that articulates design options as well as their contextual and practical implications. It assumes there is no single set of best practices in accreditation system development but, rather, an underlying set of design decisions. A "fit for purpose" approach aims to ensure that a system, policy, or program is designed and operationalized in a manner best suited to local needs and contexts. This approach is aligned with emerging models for education and international development that espouse decentralization. CONCLUSION: The framework highlights that, rather than a single best practice, variation among accreditation systems is appropriate provided that is it tailored to the needs of local contexts. Our framework is intended to provide guidance to administrators, policy-makers, and educators regarding different approaches to medical education accreditation and their applicability and appropriateness in local contexts.

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BACKGROUND: Accreditation is considered an essential ingredient for an effective system of health professions education (HPE) globally. While accreditation systems exist in various forms worldwide, there has been little written about the contemporary enterprise of accreditation and even less about its role in improving health care outcomes. We set out to 1) identify a global, contemporary definition of accreditation in the health professions, 2) describe the relationship of educational accreditation to health care outcomes, 3) identify important questions and recurring issues in twenty-first century HPE accreditation, and 4) propose a framework of essential ingredients in present-day HPE accreditation. METHODS: We identified health professions accreditation leaders via a literature search and a Google search of HPE institutions, as well as by accessing the networks of other leaders. These leaders were invited to join an international consensus consortium to advance the scholarship and thinking about HPE accreditation. We describe the consensus findings from the International Health Professions Accreditation Outcomes Consortium (IHPAOC). RESULTS: We define accreditation as the process of formal evaluation of an educational program, institution, or system against defined standards by an external body for the purposes of quality assurance and enhancement. In the context of HPE, accreditation is distinct from other forms of program evaluation or research. Accreditation can enhance health care outcomes because of its ability to influence and standardize the quality of training programs, continuously enhance curriculum to align with population needs, and improve learning environments. We describe ten fundamental and recurring elements of accreditation systems commonly found in HPE and provide an overview of five emerging developments in accreditation in the health professions based on the consensus findings. CONCLUSIONS: Accreditation has taken on greater importance in contemporary HPE. These consensus findings provide frameworks of core elements of accreditation systems and both recurring and emerging design issues. HPE scholars, educators, and leaders can build on these frameworks to advance research, development, and operation of high-quality accreditation systems worldwide.

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Juvenile-onset systemic sclerosis (jSSc) is a rare and severe autoimmune disease with associated life-threatening organ inflammation and evidence of fibrosis. The organ manifestations of jSSc resemble adult SSc, but with better outcomes and survival. The etiology of jSSc appears to reflect adult-onset SSc, with similar inflammatory mediators and autoantibodies, but with a significant population of children with uncharacterized anti-nuclear antibodies. The genetics of patients with jSSc differ from women with SSc, resembling instead the genes of adult males with SSc, with additional HLA genes uniquely associated with childhood-onset disease. Current treatments are aimed at inhibiting the inflammatory aspect of disease, but important mechanisms of fibrosis regulated by dermal white adipose tissue dendritic cells may provide an avenue for targeting and potentially reversing the fibrotic stage.

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Localized scleroderma (LS) is a complex disease characterized by a mixture of inflammation and fibrosis of the skin that, especially in the pediatric population, also affects extracutaneous tissues ranging from muscle to the central nervous system. Although developmental origins have been hypothesized, evidence points to LS as a systemic autoimmune disorder, as there is a strong correlation to family history of autoimmune disease, the presence of shared HLA types with rheumatoid arthritis, high frequency of auto-antibodies, and elevated circulating chemokines and cytokines associated with T-helper cell, IFNγ, and other inflammatory pathways. This inflammatory phenotype of the peripheral blood is reflected in the skin via microarray, RNA Sequencing and tissue staining. Research is underway to identify the key players in the pathogenesis of LS, but close approximation of inflammatory lymphocytic and macrophage infiltrate with collagen and fibroblasts deposition supports the notion that LS is a disease of inflammatory driven fibrosis. The immune system is dynamic and undergoes changes during childhood, and we speculate on how the unique features of the immune system in childhood could potentially contribute to some of the differences in LS between children and adults. Interestingly, the immune phenotype in pediatric LS resembles to some extent the healthy adult cellular phenotype, possibly supporting accelerated maturation of the immune system in LS. We discuss future directions in better understanding the pathophysiology of and how to better treat pediatric LS.

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PURPOSE: Similar to other countries, the practice of General Surgery in Canada has undergone significant evolution over the past 30 years without major changes to the training model. There is growing concern that current General Surgery residency training does not provide the skills required to practice the breadth of General Surgery in all Canadian communities and practice settings. PROCEDURE: Led by a national Task Force on the Future of General Surgery, this project aimed to develop recommendations on the optimal configuration of General Surgery training in Canada. A series of 4 evidence-based sub-studies and a national survey were launched to inform these recommendations. MAIN FINDINGS: Generalized findings from the multiple methods of the project speak to the complexity of the current practice of General Surgery: (1) General surgeons have very different practice patterns depending on the location of practice; (2) General Surgery training offers strong preparation for overall clinical competence; (3) Subspecialized training is a new reality for today's general surgeons; and (4) Generation of the report and recommendations for the future of General Surgery. A total of 4 key recommendations were developed to optimize General Surgery for the 21st century. CONCLUSIONS: This project demonstrated that a high variability of practice dependent on location contrasts with the principles of implementing the same objectives of training for all General Surgery graduates. The overall results of the project have prompted the Royal College to review the training requirements and consider a more "fit for purpose" training scheme, thus ensuring that General Surgery residency training programs would optimally prepare residents for a broad range of practice settings and locations across Canada.

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BACKGROUND: Inspection systems are used in health care to promote quality improvements, i.e. to achieve changes in organisational structures or processes, healthcare provider behaviour and patient outcomes. These systems are based on the assumption that externally promoted adherence to evidence-based standards (through inspection/assessment) will result in higher quality of health care. However, the benefits of external inspection in terms of organisational, provider and patient level outcomes are not clear. OBJECTIVES: To evaluate the effectiveness of external inspection of compliance with standards in improving healthcare organisation behaviour, healthcare professional behaviour and patient outcomes. SEARCH METHODS: We searched the following electronic databases for studies: the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, CINAHL, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effectiveness, Scopus, HMIC, Index to Theses and Intute from their inception dates up to May 2011. There was no language restriction and studies were included regardless of publication status. We searched the reference lists of included studies and contacted authors of relevant papers, accreditation bodies and the International Organization for Standardisation (ISO), regarding any further published or unpublished work. SELECTION CRITERIA: We included randomised controlled trials (RCTs), controlled clinical trials (CCTs), interrupted time-series (ITSs) and controlled before and after studies (CBAs) evaluating the effect of external inspection against external standards on healthcare organisation change, healthcare professional behaviour or patient outcomes in hospitals, primary healthcare organisations and other community-based healthcare organisations. DATA COLLECTION AND ANALYSIS: Two review authors independently applied eligibility criteria, extracted data and assessed the risk of bias of each included study. Since meta-analysis was not possible, we produced a narrative results summary. MAIN RESULTS: We identified one cluster-RCT involving 20 South African public hospitals (Salmon 2003) and one ITS involving all acute trusts in England (OPM 2009) for inclusion in this review.Salmon and colleagues (Salmon 2003) showed mixed effects of a hospital accreditation system on the compliance with COHSASA (the Council for Health Services Accreditation for South Africa) accreditation standards and eight indicators of hospital quality. Significantly improved total mean compliance score with COHSASA accreditation standards was found for 21/28 service elements: mean intervention effect (95% confidence interval (CI)) was 30% (23% to 57%) (P < 0.001). The score increased from 48% to 78% in intervention hospitals, while remaining the same in control hospitals (43%). A sub-analysis of 424 a priori identified critical criteria (19 service elements) showed significantly improved compliance with the critical standards (P < 0.001). The score increased from 41% (21% to 46%) to 75% (55% to 96%) in intervention hospitals, but was unchanged in control hospitals (37%). Only one of the nine intervention hospitals gained full accreditation status at the end of the study period, with two others reached pre-accreditation status.The median intervention effect (range) for the indicators of hospital quality of care was 2.4 (-1.9 to +11.8) and only one of the eight indicators: 'nurses perception of clinical quality, participation and teamwork' was significantly improved (mean intervention effect 5.7, P = 0.03).Re-analysis of the MRSA (methicillin-resistant Staphylococcus aureus) data showed statistically non-significant effects of the Healthcare Commissions Infection Inspection programme. AUTHORS' CONCLUSIONS: We only identified two studies for inclusion in this review, which highlights the paucity of high-quality controlled evaluations of the effectiveness of external inspection systems. No firm conclusions could therefore be drawn about the effectiveness of external inspection on compliance with standards.

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BACKGROUND: Despite widespread endorsement for administrative training during residency, teaching and learning in this area remains intermittent and limited in most programmes. AIM: To inform the development of a Manager Train-the-Trainer program for faculty, the Royal College of Physicians and Surgeons of Canada undertook a survey of perceived Manager training needs among postgraduate trainees. METHODS: A representative sample of Canadian specialty residents received a web-based questionnaire in 2009 assessing their perceived deficiencies in 13 Manager knowledge and 11 Manager skill domains, as determined by gap scores (GSs). GSs were defined as the difference between residents' perceived current and desired level of knowledge or skill in selected Manager domains. Residents' educational preferences for furthering their Manager knowledge and skills were also elicited. RESULTS: Among the 549 residents who were emailed the survey, 199 (36.2%) responded. Residents reported significant gaps in most knowledge and skills domains examined. Residents' preferred educational methods for learning Manager knowledge and skills included workshops, web-based formats and interactive small groups. CONCLUSION: The results of this national survey, highlighting significant perceived gaps in multiple Manager knowledge and skills domains, may inform the development of Manager curricula and faculty development activities to address deficiencies in training in this important area.

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Child informants routinely participate in forensic and social service investigations and are often a critical source of information. However, across research domains high levels of discrepancy between parents' reports and children's reports have been documented, which has led researchers to question children's abilities to provide accurate information about others' behavior. To date research examining parent-child discrepancies has focused on discrepancies in reports of child behavior. The aim of the present review is to examine children's abilities to provide veridical accounts of parental behaviors, drawing on developmental and clinical research to delineate factors likely to enhance or impede accuracy. Among the factors examined, age appears to have the strongest influence on the accuracy of children's reports in general. A clear distinction also emerged in the literature between children's abilities to report objective data versus information regarding abstract concepts. Although available evidence provides mixed support for the veridicality of children's reports of parenting, factors that influence children's accuracy have generally been overlooked. Namely, researchers have largely failed to discriminate between assessment of child-rearing behaviors and children's perceptions of those behaviors. The present review proposes that such failure likely accounts for a notable portion of parent-child discrepancies in reports of parenting.

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Although competency-based medical education (CBME) has attracted renewed interest in recent years among educators and policy-makers in the health care professions, there is little agreement on many aspects of this paradigm. We convened a unique partnership - the International CBME Collaborators - to examine conceptual issues and current debates in CBME. We engaged in a multi-stage group process and held a consensus conference with the aim of reviewing the scholarly literature of competency-based medical education, identifying controversies in need of clarification, proposing definitions and concepts that could be useful to educators across many jurisdictions, and exploring future directions for this approach to preparing health professionals. In this paper, we describe the evolution of CBME from the outcomes movement in the 20th century to a renewed approach that, focused on accountability and curricular outcomes and organized around competencies, promotes greater learner-centredness and de-emphasizes time-based curricular design. In this paradigm, competence and related terms are redefined to emphasize their multi-dimensional, dynamic, developmental, and contextual nature. CBME therefore has significant implications for the planning of medical curricula and will have an important impact in reshaping the enterprise of medical education. We elaborate on this emerging CBME approach and its related concepts, and invite medical educators everywhere to enter into further dialogue about the promise and the potential perils of competency-based medical curricula for the 21st century.

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At their 2009 consensus conference, the International CBME Collaborators proposed a number of central tenets of CBME in order to advance the field of medical education. Although the proposed conceptualization of CBME offers several advantages and opportunities, including a greater emphasis on outcomes, a mechanism for the promotion of learner-centred curricula, and the potential to move away from time-based training and credentialing in medicine, it is also associated with several significant barriers to adoption. This paper examines the concepts of CBME through a broad educational policy lens, identifying considerations for medical education leaders, health care institutions, and policy-makers at both the meso (program, institutional) and macro (health care system, inter-jurisdictional, and international) levels. Through this analysis, it is clear that CBME is associated with a number of complex challenges and questions, and cannot be considered in isolation from the complex systems in which it functions. Much more work is needed to engage stakeholders in dialogue, to debate the issues, and to identify possible solutions.

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OBJECTIVE: Assess the roles of care neglect and supervisory neglect, and the moderating influence of child age on childhood obesity. Study Design Child BMI, parental care neglect, and supervisory neglect were assessed in an ethnically diverse sample of 571 young children from two Midwestern States. Hierarchical linear regression was used to assess the influence of both forms of neglect and the moderating role of age. RESULTS: Fifteen percent of the children were overweight and 16.3% were obese. Care neglect significantly correlated with child BMI for younger but not older children, while supervisory neglect significantly correlated with child BMI for older but not younger children. CONCLUSIONS: The impact of two types of neglect on obesity varied across age, highlighting the importance of differentiating between types of neglectful parenting when addressing the high rate of childhood obesity in disadvantaged children.

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Child exposure to intimate partner violence (IPV) is widely acknowledged as a threat to the psycho-social and academic well-being of children. Unfortunately, as reflected in the literature, the specific link between such exposure and childhood outcomes is ambiguous. Based on a review of the literature, this article suggests that this state of affairs is due, in part, to the manner with which exposure to IPV is operationally defined. After reviewing the dominant strategies for operationally defining exposure to IPV and the problems associated with those strategies, this article reports original data contrasting three measures derived from maternal reports, three measures derived from child reports, and the limited concordance among those different indices of exposure to IPV. The implications of these findings for research on child outcomes and the clinical assessment of children who might have been exposed to IPV are discussed.

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Sturge-Weber syndrome (SWS) is characterized by capillary malformation, glaucoma, leptomeningeal vascular anomalies, and variable facial overgrowth. The purpose of this study was to document the prevalence and morbidity of facial hypertrophy in 2 cohorts: group 1, surveyed patients registered in the SWS Foundation, and group 2, patients treated at our Vascular Anomalies Center. Predictive variables included age, sex, region of capillary stain, and ocular or cerebral involvement. Outcome variables were soft-tissue and bony overgrowth, as well as the type of operative correction. In group 1, the response rate to our questionnaire was 29.3% (108/368). Facial overgrowth was documented in 60.0% of patients. Soft-tissue hypertrophy was present in 55.0%; the lip (81.0%) was the most commonly affected site. Skeletal hypertrophy was reported in 22% of patients; the maxilla (83.0%) was the most frequently overgrown bone. Overall, 23.0% of patients (36.5% with overgrowth) had an operation: 34.0% of patients with soft tissue hypertrophy and 9.0% with skeletal enlargement. In group 2, 47 patients with SWS were treated at our center: 83% had facial overgrowth, either a localized cutaneous lesion (18.0%), soft-tissue enlargement (70.0%), or bony hypertrophy (45.0%). As in group 1, the lip (75.0%) and maxilla (94.0%) were the most commonly enlarged structures. Operations were necessary for localized cutaneous lesions (86.0%), soft-tissue hypertrophy (53.0%), or skeletal overgrowth (11.0%). In conclusion, facial hypertrophy is a major component of SWS; these patients should be counseled about the risk of overgrowth and about the types of possible operative correction.

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