Improving inpatient paediatric de-labelling of allergies to beta-lactams: a quality improvement study.

OBJECTIVE: To evaluate the implementation of an antimicrobial stewardship programme-led inpatient beta-lactam allergy de-labelling programme using a direct oral provocation test (OPT). DESIGN: One-year quality improvement study using a before-after design. SETTING: Free-standing tertiary care paediatric hospital. PATIENTS: Patients with a reported beta-lactam allergy admitted to the paediatric medicine inpatient unit. INTERVENTIONS: Following standardised assessment and risk stratification of reported symptoms, patients with a low-risk history were offered an OPT. Beta-lactam allergy labels were removed if a reported history was considered non-allergic or after successful OPT. MAIN OUTCOME MEASURES: Removal of inappropriate beta-lactam allergy labels. RESULTS: 80 patients with 85 reported beta-lactam allergies were assessed. Median age was 8.1 years (IQR 4.8-12.9) and 34 (42%) were female. The majority (n=55, 69%) had an underlying medical condition. Amoxicillin was the most reported allergy (n=25, 29%). Reported reactions were primarily dermatological (n=65, 77%). Half of participants (n=40) were ineligible for OPT, with equal proportions due to clinical reasons or the nature of the reported reaction. Of the 40 eligible patients, 28 patients (70%) were de-labelled either by history alone (n=10) or OPT (n=18). All OPTs were successful. De-labelling allowed five additional patients (11% of those receiving antibiotics) to receive the preferred beta-lactam. Including patients who were subsequently assessed in the allergy clinic, almost half of all evaluated patients were de-labelled (n=37, 46%). CONCLUSIONS: An antimicrobial stewardship programme-led programme using a direct OPT was feasible and safe for expanding beta-lactam allergy de-labelling to paediatric patients admitted to the paediatric medicine inpatient unit.

Competence By Design: a transformational national model of time-variable competency-based postgraduate medical education.

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.

Strategies to Enable Transformation in Medical Education: Faculty and Trainee Development in Competence By Design.

Transformative changes in health professions education need to incorporate effective faculty development, but few very large-scale faculty development designs have been described. The Royal College of Physicians and Surgeons of Canada's Competence by Design project was launched to transform the delivery of postgraduate medical education in Canada using a competency-based model. In this paper we outline the goals, principles, and rationale of the Royal College's national strategy for faculty and resident development initiatives to support the implementation of Competence by Design. We describe the activities and resources for both faculty and trainees that facilitated the redesign of training programs for each specialty and subspecialty at the national level, as well as supporting the implementation of the redesign at the local level. This undertaking was not without its challenges: we thus reflect on those challenges, enablers, and the lessons learned, and discuss a continuous quality improvement approach that was taken to iteratively inform the implementation process moving forward.

Competency-Based Medical Education at Scale: A Road Map for Transforming National Systems of Postgraduate Medical Education.

In the past decade, the Canadian system of postgraduate medical education has been transformed with the implementation of a new approach to competency based medical education called Competence by Design. The Royal College of Physicians and Surgeons of Canada (Royal College) developed an approach to time-variable competency based medical education and adapted that design for medical, surgical, and diagnostic disciplines. New educational standards and entrustable professional activities consistent with this approach were co-created with 67 specialties and subspecialties, and implementation was scaled up across 17 universities and over 1000 postgraduate training programs. Partner engagement, systematic design of workshops to create discipline specific competency-based standards of education, and agile adaptation were all key ingredients for success. This paper describes the strategies applied by the Royal College, lessons learned regarding transformative change in the complex system of postgraduate medical education, and the current status of the Competence by Design initiative. The approach taken and lessons learned by the Royal College may be useful for other educators who are planning a transformation to CBME or any other major educational reform.

Assessing Pediatric Cephalosporin Allergic Reactions Through Direct Graded Oral Challenges.

BACKGROUND: Cephalosporins, ß-lactam antibiotics, commonly cause allergic reactions. OBJECTIVE: To assess the clinical characteristics and management of pediatric patients with suspected cephalosporin allergy using direct graded oral challenges (GOCs). METHODS: Children referred for suspected cephalosporin allergy at 4 Canadian clinics were recruited over 10 years. Data on demographics, clinical reaction characteristics, and management were collected through a questionnaire. Patients underwent a direct GOC (initially 10% of the treatment dose, then 90% after 20 min), and reactions were monitored 1 week postchallenge. Families were contacted annually for up to 5 years to detect subsequent antibiotic reactions. Logistic regression analysis identified factors associated with positive GOC reactions. RESULTS: Among the 136 patients reporting cephalosporin allergy, 75 (55.1%) were males with a median age of 3.9 years (interquartile range 2.3-8.7). Cefprozil represented the most common cephalosporin linked to the index reaction (67.6% of cases). Of the 136 direct GOCs, 5.1% had an immediate and 4.4% a nonimmediate reaction, respectively. Positive GOCs conducted in children with a history of skin-limited nonsevere rashes were classified as mild, benign skin rashes. Positive GOCs were more likely in children with food allergies (adjusted odds ratio 1.14; 95% confidence interval [95% CI] 1.00-1.29). CONCLUSIONS: Direct GOCs are safe and effective for diagnosing pediatric cases that report nonvesicular skin-limited symptoms while being treated with cephalosporins.

Providing culturally responsive care in a pediatric setting: are our trainees ready?

BACKGROUND: Extensive data consistently demonstrates inequities in access and delivery of healthcare for patients from historically marginalized populations, resulting in poorer health outcomes. To address this systemic oppression in healthcare, it is necessary to embed principles of equity, diversity, and inclusion (EDI) at an early stage within medical education. This study aimed to assess pediatric trainees' perceived interest in EDI curricula as well as their confidence in applying this knowledge to provide culturally responsive care. METHODS: An anonymous online survey was distributed to pediatric trainees at the University of Toronto. Closed-ended questions used a Likert scale to assess respondents' confidence and interest in providing culturally responsive care to patients. Open-ended questions explored trainees' perceptions of effective EDI learning modalities. A mixed methods approach was utilized, where quantitative data was summarized using descriptive statistics and descriptive content analysis was used to highlight themes within qualitative data. RESULTS: 116 pediatric trainees completed the survey, of which 72/116 (62%) were subspecialty residents/fellows and 44/116 (38%) were core residents. 97% of all responses agreed or strongly agreed that it was important to learn about providing culturally responsive care to patients from historically marginalized communities; however, many trainees lacked confidence in their knowledge of providing culturally responsive care (42%) and applying their knowledge in clinical practice (47%). Respondents identified direct clinical exposure through rotations, immersive experiences, and continuity clinics as effective EDI teaching modalities. Identified barriers included time constraints in the clinical environment, burnout, and lack of exposure to diverse patient populations. CONCLUSION: Most pediatric trainees want to provide culturally responsive care to patients from historically marginalized communities, but do not feel confident in their knowledge to do so. Trainees value learning about EDI through direct clinical exposure and immersive experiences, rather than didactic lectures or modules. These study findings will be utilized to develop and implement an enhanced EDI education curriculum for pediatric trainees at the University of Toronto and other postgraduate residency programs.

Qualitative evaluation of the contribution of CanMEDS roles in the development of area of focused competence diplomas.

Background: While many Area of Focused Competency (AFC) Diplomas are available to those who have completed Pediatric residency training, it is not known which competencies are enhanced within each AFC discipline. Our objective was to determine which CanMEDS roles were targeted by existing AFCs available to those who have completed Pediatric residency training and identify gaps within CanMEDs roles that may be fulfilled by the development of new AFCs. Methods: A qualitative study was undertaken using document analysis methodology to compare CanMEDS competencies across AFCs available to those with Royal College examination eligibility or certification in Pediatrics. RCPSC Competency Training Requirements documents were used to compare and contrast the competencies in each AFC with competencies established in Pediatric residency training. Key and Enabling Competencies were compared for each CanMEDS role to identify differences. Results: Ten AFCs were identified with eligibility requirements including Royal College examination eligibility or certification in Pediatrics. All 10 AFCs included at least one new Medical Expert competency, for a total of 42 unique competencies in this role across all AFCs. The Scholar role had only 10 new competencies across seven AFCs, while only one AFC added a single unique competency in the Collaborator role. Conclusions: The majority of new competencies contributed by AFCs lie within the CanMEDS role of Medical Expert. The Scholar and Collaborator roles have the least differences when comparing competencies of existing AFCs to those competencies established in Pediatric residency training. Developing additional AFCs that offer advanced skills in these roles may help close this gap within the discipline of Pediatrics.

Contexte: Bien que de nombreux diplômes de domaines de compétence ciblée (DCC) soient accessibles aux personnes ayant terminé leur résidence en pédiatrie, nous ne savons pas quelles sont les compétences qui sont approfondies dans chaque discipline de DCC. Notre objectif était de déterminer les rôles CanMEDS visés par les DCC actuellement accessibles aux personnes ayant terminé leur résidence en pédiatrie et de repérer les lacunes dans les rôles CanMEDS qui pourraient être comblées par l'élaboration de nouveaux DCC. Méthodes: Nous avons réalisé une étude qualitative au moyen d'analyse de documents pour comparer les compétences CanMEDS dans les DCC ouverts aux personnes admissibles à l'examen du Collège royal ou ayant une certification en pédiatrie. Les documents sur les exigences de formation du CRMCC ont été utilisés pour comparer les compétences de chaque DCC et les compétences visées dans le cadre de la formation postdoctorale en pédiatrie. Les compétences clés et les compétences habilitantes ont été comparées pour chaque rôle CanMEDS afin de repérer les différences. Résultats: Nous avons trouvé 10 DCC dont les conditions d'admissibilité comprennent l'admissibilité à l'examen du Collège royal ou la certification en pédiatrie. Chacun de ces 10 DCC comprenait au moins une nouvelle compétence d'expert médical et un total de 42 nouvelles compétences propres à ce rôle ont été répertoriées pour l'ensemble des DCC. Pour le rôle d'érudit, nous n'avons trouvé que 10 nouvelles compétences dans sept programmes de DCC, et pour celui de collaborateur, il n'y a qu'une nouvelle compétence dans un programme de DCC. Conclusions: La majorité des nouvelles compétences développées dans les DCC relèvent du rôle CanMEDS d'expert médical. Les rôles d'érudit et de collaborateur présentent le moins de différences lorsqu'on compare les compétences ciblées dans les DCC existants et celles visées dans le cadre de la formation postdoctorale en pédiatrie. La création de DCC supplémentaires permettant l'acquisition de compétences plus poussées dans ces rôles pourrait contribuer à combler cette carence au sein de la discipline de la pédiatrie.

Emerging concepts in the CanMEDS physician competency framework.

Background: The CanMEDS physician competency framework will be updated in 2025. The revision occurs during a time of disruption and transformation to society, healthcare, and medical education caused by the COVID-19 pandemic and growing acknowledgement of the impacts of colonialism, systemic discrimination, climate change, and emerging technologies on healthcare and training. To inform this revision, we sought to identify emerging concepts in the literature related to physician competencies. Methods: Emerging concepts were defined as ideas discussed in the literature related to the roles and competencies of physicians that are absent or underrepresented in the 2015 CanMEDS framework. We conducted a literature scan, title and abstract review, and thematic analysis to identify emerging concepts. Metadata for all articles published in five medical education journals between October 1, 2018 and October 1, 2021 were extracted. Fifteen authors performed a title and abstract review to identify and label underrepresented concepts. Two authors thematically analyzed the results to identify emerging concepts. A member check was conducted. Results: 1017 of 4973 (20.5%) of the included articles discussed an emerging concept. The thematic analysis identified ten themes: Equity, Diversity, Inclusion, and Social Justice; Anti-racism; Physician Humanism; Data-Informed Medicine; Complex Adaptive Systems; Clinical Learning Environment; Virtual Care; Clinical Reasoning; Adaptive Expertise; and Planetary Health. All themes were endorsed by the authorship team as emerging concepts. Conclusion: This literature scan identified ten emerging concepts to inform the 2025 revision of the CanMEDS physician competency framework. Open publication of this work will promote greater transparency in the revision process and support an ongoing dialogue on physician competence. Writing groups have been recruited to elaborate on each of the emerging concepts and how they could be further incorporated into CanMEDS 2025.

Contexte: Le référentiel de compétences CanMEDS pour les médecins sera mis à jour en 2025. Cette révision arrive à un moment où la société, les soins de santé et l'enseignement médical sont bouleversés et en pleine mutation à cause de la pandémie de la COVID-19. On est aussi à l'heure où l'on reconnaît de plus en plus les effets du colonialisme, de la discrimination systémique, des changements climatiques et des nouvelles technologies sur les soins de santé et la formation des médecins. Pour effectuer cette révision, nous avons avons extrait de la littérature scientifique les concepts émergents se rapportant aux compétences des médecins. Méthodes: Les concepts émergents ont été définis comme des idées ayant trait aux rôles et aux compétences des médecins qui sont débattues dans la littérature, mais qui sont absentes ou sous-représentées dans le cadre CanMEDS 2015. Nous avons réalisé une recherche documentaire, un examen des titres et des résumés, et une analyse thématique pour repérer les concepts émergents. Les métadonnées de tous les articles publiés dans cinq revues d'éducation médicale entre le 1er octobre 2018 et le 1er octobre 2021 ont été extraites. Quinze auteurs ont effectué un examen des titres et des résumés pour relever et étiqueter les concepts sous-représentés. Deux auteurs ont procédé à une analyse thématique des résultats pour dégager les concepts émergents. Une vérification a été faite par les membres de l'équipe. Résultats: Parmi les 4973 articles dépouillés, 1017 (20,5 %) abordaient un concept émergent. Les dix thèmes suivants sont ressortis de l'analyse thématique: l'équité, la diversité, l'inclusion et la justice sociale; l'antiracisme; l'humanité du médecin; la médecine fondée sur les données; les systèmes adaptatifs complexes; l'environnement de l'apprentissage clinique; les soins virtuels; le raisonnement clinique; l'expertise adaptative; et la santé planétaire. L'ensemble de ces thèmes ont été approuvés comme concepts émergents par l'équipe de rédaction. Conclusion: Cet examen de la littérature a permis de relever dix concepts émergents qui peuvent servir à éclairer la révision du référentiel de compétences CanMEDS pour les médecins qui aura lieu en 2025. La publication en libre accès de ce travail favorisera la transparence du processus de révision et le dialogue continu sur les compétences des médecins. Des groupes de rédaction ont été recrutés pour développer chacun des concepts émergents et pour examiner la façon dont ils pourraient être intégrés dans la version du référentiel CanMEDS de 2025.

Defining new roles and competencies for administrative staff and faculty in the age of competency-based medical education.

PURPOSE: These authors sought to define the new roles and competencies required of administrative staff and faculty in the age of CBME. METHOD: A modified Delphi process was used to define the new CBME roles and competencies needed by faculty and administrative staff. We invited international experts in CBME (volunteers from the ICBME Collaborative email list), as well as faculty members and trainees identified via social media to help us determine the new competencies required of faculty and administrative staff in the CBME era. RESULTS: Thirteen new roles were identified. The faculty-specific roles were: National Leader/Facilitator in CBME; Institutional/University lead for CBME; Assessment Process & Systems Designer; Local CBME Leads; CBME-specific Faculty Developers or Trainers; Competence Committee Chair; Competence Committee Faculty Member; Faculty Academic Coach/Advisor or Support Person; Frontline Assessor; Frontline Coach. The staff-specific roles were: Information Technology Lead; CBME Analytics/Data Support; Competence Committee Administrative Assistant. CONCLUSIONS: The authors present a new set of faculty and staff roles that are relevant to the CBME context. While some of these new roles may be incorporated into existing roles, it may be prudent to examine how best to ensure that all of them are supported within all CBME contexts in some manner.

Understanding the near-peer relationship: resident perspectives around a novel on-call workplace-based assessment.

Background: Workplace-based assessment (WBA) is a critical component of competency-based medical education (CBME), though literature on WBA for overnight call is limited. We evaluated a WBA tool completed by supervising subspecialty trainees on paediatric residents during subspecialty overnight call, for usefulness facilitating feedback/coaching in this setting. Methods: Web-based surveys were sent to residents pre- and post-WBA tool implementation monthly for four months (August-December 2018), exploring feedback frequency, Likert-scaled opinions of tool feasibility/usefulness facilitating feedback, and qualitative experiences. Assessor comments were categorized as actionable/non-actionable. Quantitative data was summarized using descriptive statistics. Qualitative data was coded to identify themes. Results: Total response rates averaged 41% (total 25 responses, average five respondents/12 residents on-call each month). Post-implementation (n = 16 responses), a non-sustained trend of increased Medical Expert feedback was observed. Residents were generally divided or disagreed on tool usefulness facilitating feedback and feasibility. Comments contained actionable feedback in < 10% of completed WBAs. Qualitative analysis revealed barriers to tool-facilitated coaching including: feedback quality and setting/environment, role of senior near-peer as assessor, interpersonal burden in encounters, and tool-specific issues. Conclusions: Increasing frequency of WBA tool completion is not sufficient to achieve CBME goals. Factors impacting feedback/coaching within the resident/near-peer dyad must be addressed.

Contexte: Tandis que l'évaluation en milieu de travail (EMT) est une composante essentielle de l'éducation médicale fondée sur les compétences (EMFC), il y a peu de recherches sur l'EMT en contexte de garde de nuit. Nous avons étudié un formulaire d'évaluation en milieu de travail rempli par des résidents en surspécialité supervisant des résidents en pédiatrie pendant la garde de nuit en surspécialité, afin de déterminer s'il facilite la rétroaction avec coaching dans ce contexte. Méthodes: Des questionnaires en ligne ont été envoyés aux résidents avant la mise en œuvre de l'outil d'EMT et à partir de celle-ci, tous les mois pendant quatre mois (d'août à décembre 2018). Ils exploraient la fréquence des rétroactions, les opinions des participants, exprimées sur une échelle de Likert, sur le caractère pratique et l'utilité de l'outil comme facilitateur de la rétroaction et leurs expériences qualitatives. Les commentaires des évaluateurs ont été catégorisés comme étant exploitables ou non exploitables. Les données quantitatives ont été résumées à l'aide de statistiques descriptives. Les données qualitatives ont été codées pour identifier les thèmes. Résultats: Le taux de réponse total était en moyenne de 41 % (total de 25 réponses, moyenne de 5 répondants/12 résidents de garde chaque mois). Après l'introduction de l'outil (n = 16 réponses), une tendance non soutenue à l'augmentation des commentaires des experts médicaux a été observée. Les résidents étaient généralement partagés ou en désaccord quant au caractère pratique de l'outil et à sa capacité à faciliter la rétroaction. Les commentaires contenaient des informations exploitables dans moins de 10 % des EMT remplies. L'analyse qualitative a révélé les obstacles suivants au fonctionnement de l'outil comme facilitateur du coaching : la qualité des commentaires et l'environnement, le rôle du presque pair senior en tant qu'évaluateur, la tension lors des rencontres de coaching et les problèmes spécifiques à l'outil. Conclusion: Pour atteindre les objectifs de l'EMFC, il ne suffit pas de remplir plus souvent l'outil d'EMT. Les facteurs qui influencent la rétroaction avec coaching au sein de la dyade résident-presque pair doivent également être pris en compte.

Food protein-induced enterocolitis syndrome in a tertiary pediatric center: safety of guideline-conforming food challenges.

Food protein-induced enterocolitis syndrome is a non-IgE-mediated reaction to food that is poorly understood, and underdiagnosed. Trigger foods can belong to any food group, but are most commonly milk, soy, rice, oat, egg, and fish. In this retrospective study (2015-2020), we describe the clinical presentations and triggers of 37 children referred to tertiary hospital with a confirmed or suspected diagnosis of food protein-inducted enterocolitis. We reviewed the safety of current recommendations by looking at the outcome of 24 oral food challenges. All of these patients presented with clear cut systemic reactions including lethargy. We also assessed the severity of the reactions. Oral food challenges occurred in the hospital day unit with the majority of patients having IV access in place. Despite a clear history of FPIES with lethargy and the requirement for re-hydration of the challenged population, 21/24 (88%) of the FPIES OFCs were successful. Of the three patients who reacted, symptoms were of moderate nature, mainly vomiting. This highlights the importance of early diagnosis and a pro-active approach to performing guideline-directed oral food challenges in patients with food protein-induced enterocolitis syndrome.

Assessing nephrology competence in general paediatrics-A survey of general paediatricians, paediatric nephrologists, residents, and program directors.

Objective: The objective of this study was to identify nephrology topics of lowest perceived competency and importance for general paediatricians. Methods: Surveys were distributed to general paediatricians, paediatric residents, paediatric residency program directors, and paediatric nephrologists. Perceived importance and competence were rated on a 5-point Likert scale. Means and 95% confidence intervals were calculated. Results: Mean perceived competency from general paediatricians across all nephrology domains was 3.0, 95%CI (2.9 to 3.1) and mean importance was 3.2, 95%CI (3.1 to 3.3). Domains scoring below the means for competence and importance, respectively were kidney stones (2.5, 95%CI [2.2 to 2.7]) and 2.6, 95%CI [2.3 to 2.8]), acute kidney injury (2.5, 95%CI [2.2 to 2.8] and 2.4, 95%CI [2.1 to 2.8]), chronic kidney disease (1.9, 95%CI [1.7 to 2.2] and 2.1, 95%CI [1.8 to 2.4]), tubular disorders (1.8, 95%CI [1.6 to 2.0] and 2.0, 95%CI [1.8 to 2.3]), and kidney transplant (1.6, 95%CI [1.4 to 1.8] and 1.7, 95%CI [1.4 to 1.9]). Residents, program directors, and paediatric nephrologists agreed that stones, chronic kidney disease, tubular disorders, and transplant were of lower importance. However, acute kidney injury was the domain with the largest discrepancy in perceived importance between residents (4.4, 95%CI [4.2 to 4.6]), nephrologists (4.2, 95%CI [3.8 to 4.6]), and program directors (4.2, 95%CI [3.7 to 4.7]) compared to general paediatricians ([2.4, 95%CI [2.1 to 2.8]; P<0.05). Conclusion: Paediatricians did not believe acute kidney injury was important to their practice, despite expert opinion and evidence of long-term consequences. Educational interventions must address deficits in crucial domains of renal health in paediatrics.

Dietary exposures and allergy prevention in high-risk infants.

Infants at high risk for developing a food allergy have either an atopic condition (such as eczema) themselves or an immediate family member with such a condition. Breastfeeding should be promoted and supported regardless of issues pertaining to food allergy prevention, but for infants whose mothers cannot or choose not to breastfeed, using a specific formula (i.e., hydrolyzed formula) is not recommended to prevent food allergies. When cow's milk protein formula has been introduced in an infant's diet, make sure that regular ingestion (as little as 10 mL daily) is maintained to prevent loss of tolerance. For high-risk infants, there is compelling evidence that introducing allergenic foods early-at around 6 months, but not before 4 months of age-can prevent common food allergies, and allergies to peanut and egg in particular. Once an allergenic food has been introduced, regular ingestion (e.g., a few times a week) is important to maintain tolerance. Common allergenic foods can be introduced without pausing for days between new foods, and the risk for a severe reaction at first exposure in infancy is extremely low. Pre-emptive in-office screening before introducing allergenic foods is not recommended. No recommendations can be made at this time about the role of maternal dietary modification during pregnancy or lactation, or about supplementing with vitamin D, omega 3, or pre- or probiotics as means to prevent food allergy.

Feedback and coaching.

If used thoughtfully and with intent, feedback and coaching will promote learning and growth as well as personal and professional development in our learners. Feedback is an educational tool as well as a social interaction between learner and supervisor, in the context of a respectful and trusting relationship. It challenges the learner's thinking and supports the learner's growth. Coaching is an educational philosophy dedicated to supporting learners' personal and professional development and growth and supporting them to reach their potential. In clinical education, feedback is most effective when it is explicitly distinguished from summative assessment. Importantly, feedback should be about firsthand observed behaviors (which can be direct or indirect) and not about information which comes from a third party. Learners are more receptive to feedback if it comes from a source that they perceive as credible, and with whom they have developed rapport. The coaching relationship between learner and supervisor should also be built on mutual trust and respect. Coaching can be provided in the moment (feedback on everyday clinical activities that leads to performance improvement, even with short interaction with a supervisor) and over time (a longer term relationship with a supervisor in which there is reflection on the learner's development and co-creation of new learning goals). Feedback and coaching are most valuable when the learner and teacher exhibit a growth mindset. At the organizational level, it is important that both the structures and training are in place to ensure a culture of effective feedback and coaching in the clinical workplace.Conclusions: Having a thoughtful and intentional approach to feedback and coaching with learners, as well as applying evidence-based principles, will not only contribute in a significant way to their developmental progression, but will also provide them with the tools they need to have the best chance of achieving competence throughout their training. What is Known: • Feedback and coaching are key to advancing the developmental progression of trainees as they work towards achieving competence. • Feedback is not a one-way delivery of specific information from supervisor to trainee, but rather a social interaction between two individuals in which trust and respect play a key role. • Provision of effective feedback may be hampered by confusing formative (supporting trainee learning and development) and summative (the judgment that is made about a trainee's level of competence) purposes. What is New: • Approaches to both the provision of feedback/coaching and the assessment of competence must be developed in parallel to ensure success in clinical training programs. • Faculty development is essential to provide clinical teachers with the skills to provide effective feedback and coaching. • Coaching's effectiveness relies on nurturing strong trainee-supervisor relationships, ensuring high-quality feedback, nourishing a growth mindset, and encouraging an institutional culture that embraces feedback and coaching.

Homozygous duplication identified by whole genome sequencing causes LRBA deficiency.

In more than one-third of primary immunodeficiency (PID) patients, extensive genetic analysis including whole-exome sequencing (WES) fails to identify the genetic defect. Whole-genome sequencing (WGS) is able to detect variants missed by other genomics platforms, enabling the molecular diagnosis of otherwise unresolved cases. Here, we report two siblings, offspring of consanguineous parents, who experienced similar severe events encompassing early onset of colitis, lymphoproliferation, and hypogammaglobulinemia, typical of lipopolysaccharide-responsive and beige-like anchor (LRBA) or cytotoxic T lymphocyte antigen 4 (CTLA4) deficiencies. Gene-panel sequencing, comparative genomic hybridization (CGH) array, and WES failed to reveal a genetic aberration in relevant genes. WGS of these patients detected a 12.3 kb homozygous tandem duplication that was absent in control cohorts and is predicted to disrupt the reading frame of the LRBA gene. The variant was validated by PCR and Sanger sequencing, demonstrating the presence of the junction between the reference and the tandem-duplicated sequence. Droplet digital PCR (ddPCR) further confirmed the copy number in the unaffected parents (CN = 3, heterozygous) and affected siblings (CN = 4, homozygous), confirming the expected segregation pattern. In cases of suspected inherited immunodeficiency, WGS may reveal a mutation when other methods such as microarray and WES analysis failed to detect an aberration.

Safety of administration of BNT162b2 mRNA (Pfizer-BioNTech) COVID-19 vaccine in youths and young adults with a history of acute lymphoblastic leukemia and allergy to PEG-asparaginase.

Vaccinationis a critical tool in the prevention of COVID-19 infection for individuals and for communities. The mRNA vaccines contain polyethylene glycol (PEG) as a stabilizer. Currently, in North America, only the BNT162b2 (Pfizer-BioNTech) mRNA vaccine is approved for individuals aged 12-17. Most patients treated with contemporary regimens for acute lymphoblastic leukemia receive PEG-asparaginase (PEG-ASNase) and 10%-30% will develop allergic reactions. Optimizing access and safety for vaccine administration for these patients is critical. This report describes a process developed to support COVID vaccination in a cohort of adolescents and young adults with a history of PEG-ASNase allergy.

Establishing Amoxicillin Allergy in Children Through Direct Graded Oral Challenge (GOC): Evaluating Risk Factors for Positive Challenges, Safety, and Risk of Cross-Reactivity to Cephalosporines.

BACKGROUND: Data on the diagnostic properties of direct oral challenges without the use of skin tests in children with suspected amoxicillin allergy are sparse. OBJECTIVE: Assess the use of direct oral challenges. METHODS: A cohort study was conducted between March 2013 and March 2020, in Montreal and Winnipeg. All children referred with reported history of benign reactions (ie, limited to the skin with no mucosal lesions and no vesicles) to amoxicillin were recruited and a 2-step graded oral challenge (GOC) was conducted. Data were collected on demographic characteristics, clinical characteristics, and comorbidities. Eligible children were followed to assess reactions to subsequent use of amoxicillin and to assess the safety of cephalexin use in children with a positive GOC. RESULTS: Among 1914 children recruited, 1811 (94.6%) tolerated the GOC, 42 (2.2%) developed mild immediate reactions, and 61 (3.2%) developed mild nonimmediate reactions. Among 265 participants who had a negative GOC and reused amoxicillin, 226 (85.3%) reported tolerance and 39 (14.7%) had mild cutaneous reactions. Chronic urticaria (adjusted odds ratio [aOR], 1.16; 95% CI, 1.09-1.23) and an index reaction occurring within 5 minutes of exposure (aOR, 1.09; 95% CI, 1.04-1.14) were associated with immediate reactions during the GOC. Symptoms lasting longer than 7 days (aOR, 1.05; 95% CI, 1.02-1.09) and parental drug hypersensitivity (aOR, 1.04; 95% CI, 1.03-1.06) were associated with nonimmediate reactions. Among those reacting to the GOC, 12.5% reacted with mild cutaneous reactions to cephalexin challenge. CONCLUSIONS: Direct GOCs are an accurate and safe confirmatory to establish true hypersensitivity among children reporting benign reactions to amoxicillin.

Progressive decline of T and B cell numbers and function in a patient with CDC42 deficiency.

Single allele mutations in the Cell Division Control protein 42 homolog (CDC42) gene were recently shown to cause Takenouchi-Kosaki syndrome with diverse manifestations. These include persistent mild thrombocytopenia with large platelet size, severe developmental delay, growth retardation, facial dysmorphism, and other neurodevelopmental and hematological anomalies. CDC42 deficiency might also cause myelofibrosis, myeloproliferation, and severe autoinflammation. CDC42 closely interacts with the Wiskott-Aldrich Syndrome Protein, but little is still known about the immune abnormalities associated with CDC42 deficiency. Detailed immune evaluations were performed in a patient diagnosed with a CDC42 Tyr64Cys mutation. The 19-year-old female suffered from recurrent pneumonia, otitis media, and bacteremia, which resolved at 10 years of age, concordant with the initiation of amoxicillin prophylaxis. In addition, the patient had frequent viral upper respiratory tract infections, which resolved without need for medical interventions. Immune evaluations demonstrated decreased immunoglobulin levels, inability to maintain antibody responses, progressive decline in the number of CD19+ B cells, and decreased switched memory B cells. There was also a decrease in CD4+ and CD8+ T cells, markedly reduced naïve T cells, and intermittent depressed proliferation of T cells to stimulation. Natural killer cells' number and functions were normal. However, no opportunistic infections were observed, nor was there evidence for autoinflammation. CDC42 deficiency might also be associated with decline in T and B cell function. Therefore, immunity in patients with CDC42 defects should be closely monitored, particularly among those with frequent infections or systemic autoinflammation.