Publications by authors named "Frenkel J"

Background: Much is known about the positive effects of teachers' self-efficacy on instruction and student outcomes, but the processes underlying these relations are unknown.

Aims: We aimed to examine the effects of teacher self-efficacy for student engagement (TSESE) before a lesson on teachers' nonverbal immediacy (NVI) and their enthusiastic teaching. Furthermore, we examined how NVI and enthusiastic teaching affected students' interest after the lesson, controlling for prior interest.

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Mevalonate kinase deficiency (MKD), a rare auto-inflammatory disorder, arises from mutations in the gene, disrupting isoprenoid biosynthesis, and affecting cellular processes. This comprehensive review provides an updated perspective on MKD, including its aetiology, pathogenesis, diagnostic modalities, and therapeutic strategies. Based on recent research and clinical advances, our objective is to bridge the knowledge gaps in the 2015 SHARE guidelines.

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Online education has become increasingly popular in recent years, and video lectures have emerged as a common instructional format. While the importance of instructors' nonverbal social cues such as gaze, facial expression, and gestures for learning progress in face-to-face teaching is well-established, their impact on instructional videos is not fully understood. Most studies on nonverbal social cues in instructional videos focus on isolated cues rather than considering multimodal nonverbal behavior patterns and their effects on the learning progress.

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Background: Developing theoretical courses for post-graduate medical training that are aligned to current workplace-based learning practices and adaptive to change in the field is challenging, especially in (sub) specialties where time for re-design is limited and needs to be performed while education continues.

Approach: An instructional design method was applied based on flexible co-design to improve post-graduate theoretical courses in child and adolescent psychiatry (CAP) in the Netherlands. In four phases over a period of three years, courses were re-designed at a national level.

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Background: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries.

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Article Synopsis
  • - The study assessed safety events in patients with Autoinflammatory diseases using data from the Eurofever registry, focusing on adverse events (AEs) reported since 2009.
  • - A total of 2464 patients were included in the analysis, highlighting that 479 AEs were reported, with serious AEs and drug-related AEs being particularly noted, especially in relation to biologic DMARDs and colchicine.
  • - The findings underscore the need for consistent monitoring of AEs in rare conditions to better understand the safety of treatments.
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Introduction: Despite its high potential, patient feedback does not always result in learning. For feedback to be effective students must engage with it, which partly depends on their perceptions of feedback. To better understand student engagement with patient feedback in a clinical context, this study explored the following research questions: 1) What are medical students' general beliefs about patient feedback and what are their specific perceptions of feedback messages? 2) What is the difference between these general beliefs and feedback message perceptions before and after patient feedback training?

Methods: The study context was a 12-week clerkship combining Pediatrics and Gynecology, which included feedback training for students and asking for patient feedback.

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Introduction: Given the complexity of teaching clinical reasoning to (future) healthcare professionals, the utilization of serious games has become popular for supporting clinical reasoning education. This scoping review outlines games designed to support teaching clinical reasoning in health professions education, with a specific emphasis on their alignment with the 8-step clinical reasoning cycle and the reflective practice framework, fundamental for effective learning.

Methods: A scoping review using systematic searches across seven databases (PubMed, CINAHL, ERIC, PsycINFO, Scopus, Web of Science, and Embase) was conducted.

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Introduction: In pediatric education, caregivers are increasingly involved to share their perspective. Yet, an in-depth understanding of the perspective-taking process between medical students and caregivers is lacking. This study explored: 1) Which strategies do medical students use to take a caregiver's perspective and which facilitators and constraints do they perceive? 2) Which strategies do caregivers use to share their perspective with students? and 3) How do students' perspective-taking strategies relate to caregivers' perspective-sharing strategies?

Methods: In an online lesson: two caregivers of pediatric patients, shared their story with 27 fourth-year Dutch medical students.

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Article Synopsis
  • - The study aimed to compare the clinical features and treatment responses of patients with the TNFRSF1A-pR92Q variant to those with classical TRAPS, PFAPA, and SURF, using data from the Eurofever registry.
  • - 361 patients were analyzed, revealing that pR92Q variant patients typically had an older onset of disease and shared more similarities with SURF patients, while classical TRAPS patients exhibited distinct traits like migratory rash and familial history.
  • - Despite some differences in symptoms, all patient groups responded similarly to treatment, with steroids being the most commonly prescribed and effective; this suggests the need for a revised approach to diagnosing and managing pR92Q variant patients.
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Introduction: Patient feedback is becoming ever more important in medical education. Whether students engage with feedback is partly determined by how credible they think the feedback provider is. Despite its importance for feedback engagement, little is known about how medical students judge the credibility of patients.

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Mevalonate kinase (MK) deficiency is a rare autosomal recessive metabolic disorder caused by pathogenic variants in the gene with a broad phenotypic spectrum including autoinflammation, developmental delay and ataxia. Typically, neurological symptoms are considered to be part of the severe end of the phenotypical spectrum and are reported to be in addition to the autoinflammatory symptoms. Here, we describe a patient with MK deficiency with severe neurological symptoms but without autoinflammation and we found several similar patients in the literature.

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Moving towards person-centered care, with equal partnership between healthcare professionals and patients, requires a solid role for the patient in the education of students and professionals. Patients can be involved as teachers, assessors, curriculum developers, and policy-makers. Yet, many of the initiatives with patients are isolated, small events for targeted groups and there is a lack of patient involvement at the institutional level.

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Endophthalmitis is a bacterial or fungal infection in the deep spaces of the eye. The diagnosis of endophthalmitis has traditionally been made by vitreous humor culture and is commonly missed on initial presentation. In this case report, we emphasize the role of ocular point-of-care ultrasound (POCUS) in revealing endophthalmitis as a primary differential diagnosis for a patient presenting with unilateral eye pain and significant swelling that limited physical examination of the eye.

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Objectives: Mevalonate kinase deficiency (MKD) is a rare autoinflammatory syndrome. Several reports have described allogeneic hematopoietic stem cell transplantation in severely affected patients, sometimes with promising results. In view of the scarcity of data, this study aims to analyse the efficacy and safety of allogeneic hematopoietic stem cell transplantation (HSCT) to give a more complete overview of this treatment.

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Objective: To prepare medical students for a rapidly changing healthcare landscape, where new means of communication emerge, innovative teaching methods are needed. We developed a project-based learning course in which medical students design audiovisual patient information in collaboration with patients and with students in Communication and Information Sciences (CIS). We studied what learning mechanisms are triggered in medical students by elements of a project-based-learning course.

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Article Synopsis
  • IL-1 mediated systemic autoinflammatory diseases, such as CAPS and TRAPS, are rare inflammatory conditions predominantly affecting children, associated with severe symptoms and poor outcomes if left untreated.
  • A multinational task force of experts created evidence-based recommendations to standardize the diagnosis, treatment, and monitoring of these diseases to improve patient care.
  • The task force established key principles and specific guidelines covering diagnosis, therapy, and long-term monitoring to better manage organ damage and the overall treatment of affected patients.
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Article Synopsis
  • IL-1 mediated systemic autoinflammatory diseases like CAPS, TRAPS, MKD, and DIRA primarily affect young children, showing various organ involvement, and require effective treatment strategies due to their severe nature and poor prognosis if untreated.* -
  • A multidisciplinary task force was created to establish evidence-based guidelines for diagnosing and managing these diseases, using systematic reviews and expert consensus methods.* -
  • The task force created comprehensive recommendations, including 5 guiding principles, 14 statements on diagnosis, 10 on treatment, and 9 on long-term monitoring, aiming to improve patient outcomes through standardized care.*
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Objectives: To evaluate the long-term efficacy and safety of canakinumab in patients with mevalonate kinase deficiency during the open label extension (weeks 41-113) of the randomized controlled CLUSTER trial.

Methods: During a 72-week period, patients received open-label canakinumab 150 or 300 mg, every 4 or 8 weeks. The disease activity was evaluated every 8 weeks using physician global assessment and counting the number of flares.

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Background: Although a number of successful handoff interventions have been reported, the handoff process remains vulnerable because it relies on memory. The aim of this study was to investigate the effect of deliberate cognitive processing (i.e.

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Background: TNF receptor-associated periodic syndrome (TRAPS) is a rare autoinflammatory disease caused by dominant mutation of the TNF super family receptor 1A (TNFRSF1A) gene. Data regarding long-term treatment outcomes are lacking.

Objective: To assess correlations of genotype-phenotypes in patients with TRAPS, as defined by the International Study Group for Systemic Autoinflammatory Diseases (INSAID) classification and Eurofever criteria, with treatment responses.

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Patients with type 2 diabetes (T2D) have a lower risk of Mycobacterium tuberculosis infection, progression from infection to tuberculosis (TB) disease, TB morality and TB recurrence, when being treated with metformin. However, a detailed mechanistic understanding of these protective effects is lacking. Here, we use mass cytometry to show that metformin treatment expands a population of memory-like antigen-inexperienced CD8CXCR3 T cells in naive mice, and in healthy individuals and patients with T2D.

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Tuberculosis (TB) is a chronic inflammatory disease that is often associated with alterations in systemic and cellular metabolism that resolves following successful antimicrobial drug treatment. We hypothesized that altered systemic glucose metabolism as a consequence of Mycobacterium tuberculosis (Mtb) infection, contributes to TB pathogenesis, and when normalized with anti-glycemic drugs would improve clinical outcomes. To test this hypothesis, guinea pigs were treated daily with the anti-diabetic drug metformin starting 4 weeks prior or concurrent with aerosol exposure to the H37Rv strain of Mtb.

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IL-1 mediated auto-inflammatory diseases are characterised by episodes of unexplained fever, generalized and localized inflammation. The characteristic symptoms predominantly result from exaggerated activation of innate immune pathways. However, in some patients with typical IL-1 mediated diseases, chronic disease manifestations develop in the absence of acute inflammation, suggesting the involvement of adaptive immune pathways.

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Mevalonate kinase deficiency (MKD) is an inborn error of metabolism leading to a syndrome characterized by recurrent inflammation. This clinically manifests itself as fever and can be accompanied by gastrointestinal symptoms, oral ulcers, cervical lymphadenopathy, and skin rash. We searched Pubmed, Embase, Cochrane, and CINAHL for relevant articles.

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